-
1
-
-
0033525788
-
Mitochondrial evolution
-
Gray M. W., Burger G., Lang B. F., Mitochondrial evolution Science 1999 283 5407 1476 1481
-
(1999)
Science
, vol.283
, Issue.5407
, pp. 1476-1481
-
-
Gray, M.W.1
Burger, G.2
Lang, B.F.3
-
2
-
-
0030046495
-
Mitochondria and diabetes: Genetic, biochemical, and clinical implications of the cellular energy circuit
-
Gerbitz K.-D., Gempel K., Brdiczka D., Mitochondria and diabetes: genetic, biochemical, and clinical implications of the cellular energy circuit Diabetes 1996 45 2 113 126
-
(1996)
Diabetes
, vol.45
, Issue.2
, pp. 113-126
-
-
Gerbitz, K.-D.1
Gempel, K.2
Brdiczka, D.3
-
3
-
-
50449084335
-
Mitochondrial dysfunction in type 2 diabetes and obesity
-
Hojlund K., Mogensen M., Sahlin K., Beck-Nielsen H., Mitochondrial dysfunction in type 2 diabetes and obesity Endocrinology and Metabolism Clinics of North America 2008 37 3 713 731
-
(2008)
Endocrinology and Metabolism Clinics of North America
, vol.37
, Issue.3
, pp. 713-731
-
-
Hojlund, K.1
Mogensen, M.2
Sahlin, K.3
Beck-Nielsen, H.4
-
4
-
-
34250624810
-
A cell biological perspective on mitochondrial dysfunction in Parkinson disease and other neurodegenerative diseases
-
Mandemakers W., Morais V. A., De Strooper B., A cell biological perspective on mitochondrial dysfunction in Parkinson disease and other neurodegenerative diseases Journal of Cell Science 2007 120 10 1707 1716
-
(2007)
Journal of Cell Science
, vol.120
, Issue.10
, pp. 1707-1716
-
-
Mandemakers, W.1
Morais, V.A.2
De Strooper, B.3
-
5
-
-
33745274726
-
Mitochondria: Dynamic organelles in disease, aging, and development
-
Chan D. C., Mitochondria: dynamic organelles in disease, aging, and development Cell 2006 125 7 1241 1252
-
(2006)
Cell
, vol.125
, Issue.7
, pp. 1241-1252
-
-
Chan, D.C.1
-
6
-
-
38649132337
-
Mitochondriaa nexus for aging, calorie restriction, and sirtuins?
-
Guarente L., Mitochondriaa nexus for aging, calorie restriction, and sirtuins? Cell 2008 132 2 171 176
-
(2008)
Cell
, vol.132
, Issue.2
, pp. 171-176
-
-
Guarente, L.1
-
7
-
-
9644274004
-
The epidemiology of mitochondrial disorderspast, present and future
-
Schaefer A. M., Taylor R. W., Turnbull D. M., Chinnery P. F., The epidemiology of mitochondrial disorderspast, present and future Biochimica et Biophysica Acta 2004 1659 2-3 115 120
-
(2004)
Biochimica et Biophysica Acta
, vol.1659
, Issue.23
, pp. 115-120
-
-
Schaefer, A.M.1
Taylor, R.W.2
Turnbull, D.M.3
Chinnery, P.F.4
-
8
-
-
0033525924
-
Oxidative phosphorylation at the fin de siecle
-
Saraste M., Oxidative phosphorylation at the fin de siecle Science 1999 283 5407 1488 1493
-
(1999)
Science
, vol.283
, Issue.5407
, pp. 1488-1493
-
-
Saraste, M.1
-
9
-
-
0033967568
-
Isolated complex i deficiency in children: Clinical, biochemical and genetic aspects
-
Loeffen J. L. C. M., Smeitink J. A. M., Trijbels J. M. F., Isolated complex I deficiency in children: clinical, biochemical and genetic aspects Human Mutation 2000 15 2 123 134
-
(2000)
Human Mutation
, vol.15
, Issue.2
, pp. 123-134
-
-
Loeffen, J.L.C.M.1
Smeitink, J.A.M.2
Trijbels, J.M.F.3
-
11
-
-
4944260285
-
Mitochondrial disorders
-
Zeviani M., Di Donato S., Mitochondrial disorders Brain 2004 127 10 2153 2172
-
(2004)
Brain
, vol.127
, Issue.10
, pp. 2153-2172
-
-
Zeviani, M.1
Di Donato, S.2
-
13
-
-
39649120348
-
Inherited mitochondrial diseases of DNA replication
-
Copeland W. C., Inherited mitochondrial diseases of DNA replication Annual Review of Medicine 2008 59 131 146
-
(2008)
Annual Review of Medicine
, vol.59
, pp. 131-146
-
-
Copeland, W.C.1
-
14
-
-
2442691791
-
Missense mutation in pseudouridine synthase 1 (PUS1) causes mitochondrial myopathy and sideroblastic anemia (MLASA)
-
Bykhovskaya Y., Casas K., Mengesha E., Inbal A., Fischel-Ghodsian N., Missense mutation in pseudouridine synthase 1 (PUS1) causes mitochondrial myopathy and sideroblastic anemia (MLASA) American Journal of Human Genetics 2004 74 6 1303 1308
-
(2004)
American Journal of Human Genetics
, vol.74
, Issue.6
, pp. 1303-1308
-
-
Bykhovskaya, Y.1
Casas, K.2
Mengesha, E.3
Inbal, A.4
Fischel-Ghodsian, N.5
-
15
-
-
8344259033
-
Mutant mitochondrial elongation factor G1 and combined oxidative phosphorylation deficiency
-
Coenen M. J. H., Antonicka H., Ugalde C., Mutant mitochondrial elongation factor G1 and combined oxidative phosphorylation deficiency New England Journal of Medicine 2004 351 20 2080 2086
-
(2004)
New England Journal of Medicine
, vol.351
, Issue.20
, pp. 2080-2086
-
-
Coenen, M.J.H.1
Antonicka, H.2
Ugalde, C.3
-
16
-
-
35348983348
-
Deleterious mutation in the mitochondrial arginyl-transfer RNA synthetase gene is associated with pontocerebellar hypoplasia
-
Edvardson S., Shaag A., Kolesnikova O., Deleterious mutation in the mitochondrial arginyl-transfer RNA synthetase gene is associated with pontocerebellar hypoplasia American Journal of Human Genetics 2007 81 4 857 862
-
(2007)
American Journal of Human Genetics
, vol.81
, Issue.4
, pp. 857-862
-
-
Edvardson, S.1
Shaag, A.2
Kolesnikova, O.3
-
17
-
-
34147144142
-
Nonsense mutation in pseudouridylate synthase 1 (PUS1) in two brothers affected by myopathy, lactic acidosis and sideroblastic anaemia (MLASA)
-
Fernandez-Vizarra E., Berardinelli A., Valente L., Tiranti V., Zeviani M., Nonsense mutation in pseudouridylate synthase 1 (PUS1) in two brothers affected by myopathy, lactic acidosis and sideroblastic anaemia (MLASA) Journal of Medical Genetics 2007 44 3 173 180
-
(2007)
Journal of Medical Genetics
, vol.44
, Issue.3
, pp. 173-180
-
-
Fernandez-Vizarra, E.1
Berardinelli, A.2
Valente, L.3
Tiranti, V.4
Zeviani, M.5
-
18
-
-
9144268494
-
Defective mitochondrial translation caused by a ribosomal protein (MRPS16) mutation
-
Miller C., Saada A., Shaul N., Defective mitochondrial translation caused by a ribosomal protein (MRPS16) mutation Annals of Neurology 2004 56 5 734 738
-
(2004)
Annals of Neurology
, vol.56
, Issue.5
, pp. 734-738
-
-
Miller, C.1
Saada, A.2
Shaul, N.3
-
19
-
-
37249071299
-
Antenatal mitochondrial disease caused by mitochondrial ribosomal protein (MRPS22) mutation
-
Saada A., Shaag A., Arnon S., Antenatal mitochondrial disease caused by mitochondrial ribosomal protein (MRPS22) mutation Journal of Medical Genetics 2007 44 12 784 786
-
(2007)
Journal of Medical Genetics
, vol.44
, Issue.12
, pp. 784-786
-
-
Saada, A.1
Shaag, A.2
Arnon, S.3
-
20
-
-
34047109743
-
Mitochondrial aspartyl-tRNA synthetase deficiency causes leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation
-
Scheper G. C., van der Klok T., van Andel R. J., Mitochondrial aspartyl-tRNA synthetase deficiency causes leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation Nature Genetics 2007 39 4 534 539
-
(2007)
Nature Genetics
, vol.39
, Issue.4
, pp. 534-539
-
-
Scheper, G.C.1
Van Der Klok, T.2
Van Andel, R.J.3
-
21
-
-
33751085653
-
Distinct clinical phenotypes associated with a mutation in the mitochondrial translation elongation factor EFTs
-
Smeitink J. A. M., Elpeleg O., Antonicka H., Distinct clinical phenotypes associated with a mutation in the mitochondrial translation elongation factor EFTs American Journal of Human Genetics 2006 79 5 869 877
-
(2006)
American Journal of Human Genetics
, vol.79
, Issue.5
, pp. 869-877
-
-
Smeitink, J.A.M.1
Elpeleg, O.2
Antonicka, H.3
-
22
-
-
33846006253
-
Infantile encephalopathy and defective mitochondrial DNA translation in patients with mutations of mitochondrial elongation factors EFG1 and EFTu
-
Valente L., Tiranti V., Marsano R. M., Infantile encephalopathy and defective mitochondrial DNA translation in patients with mutations of mitochondrial elongation factors EFG1 and EFTu American Journal of Human Genetics 2007 80 1 44 58
-
(2007)
American Journal of Human Genetics
, vol.80
, Issue.1
, pp. 44-58
-
-
Valente, L.1
Tiranti, V.2
Marsano, R.M.3
-
23
-
-
74549201114
-
DARS2 mutations in mitochondrial leucoencephalopathy and multiple sclerosis
-
Isohanni P., Linnankivi T., Buzkova J., DARS2 mutations in mitochondrial leucoencephalopathy and multiple sclerosis Journal of Medical Genetics 2010 47 1 66 70
-
(2010)
Journal of Medical Genetics
, vol.47
, Issue.1
, pp. 66-70
-
-
Isohanni, P.1
Linnankivi, T.2
Buzkova, J.3
-
24
-
-
69649100936
-
Acute infantile liver failure due to mutations in the TRMU gene
-
Zeharia A., Shaag A., Pappo O., Acute infantile liver failure due to mutations in the TRMU gene American Journal of Human Genetics 2009 85 3 401 407
-
(2009)
American Journal of Human Genetics
, vol.85
, Issue.3
, pp. 401-407
-
-
Zeharia, A.1
Shaag, A.2
Pappo, O.3
-
25
-
-
33744752749
-
The molecular basis for tissue specificity of the oxidative phosphorylation deficiencies in patients with mutations in the mitochondrial translation factor EFG1
-
Antonicka H., Sasarman F., Kennaway N. G., Shoubridge E. A., The molecular basis for tissue specificity of the oxidative phosphorylation deficiencies in patients with mutations in the mitochondrial translation factor EFG1 Human Molecular Genetics 2006 15 11 1835 1846
-
(2006)
Human Molecular Genetics
, vol.15
, Issue.11
, pp. 1835-1846
-
-
Antonicka, H.1
Sasarman, F.2
Kennaway, N.G.3
Shoubridge, E.A.4
-
26
-
-
0019423856
-
Sequence and organization of the human mitochondrial genome
-
Anderson S., Bankier A. T., Barrell B. G., Sequence and organization of the human mitochondrial genome Nature 1981 290 5806 457 465
-
(1981)
Nature
, vol.290
, Issue.5806
, pp. 457-465
-
-
Anderson, S.1
Bankier, A.T.2
Barrell, B.G.3
-
28
-
-
33645214213
-
Mutation pressure and the evolution of organelle genomic architecture
-
Lynch M., Koskella B., Schaack S., Mutation pressure and the evolution of organelle genomic architecture Science 2006 311 5768 1727 1730
-
(2006)
Science
, vol.311
, Issue.5768
, pp. 1727-1730
-
-
Lynch, M.1
Koskella, B.2
Schaack, S.3
-
30
-
-
17744393686
-
Mitochondrial DNA mutations in human disease
-
Taylor R. W., Turnbull D. M., Mitochondrial DNA mutations in human disease Nature Reviews Genetics 2005 6 5 389 402
-
(2005)
Nature Reviews Genetics
, vol.6
, Issue.5
, pp. 389-402
-
-
Taylor, R.W.1
Turnbull, D.M.2
-
31
-
-
0017758576
-
Mouse l cell mitochondrial DNA molecules are selected randomly for replication throughout the cell cycle
-
Bogenhagen D., Clayton D. A., Mouse l cell mitochondrial DNA molecules are selected randomly for replication throughout the cell cycle Cell 1977 11 4 719 727
-
(1977)
Cell
, vol.11
, Issue.4
, pp. 719-727
-
-
Bogenhagen, D.1
Clayton, D.A.2
-
32
-
-
0019978703
-
Replication of animal mitochondrial DNA
-
Clayton D. A., Replication of animal mitochondrial DNA Cell 1982 28 4 693 705
-
(1982)
Cell
, vol.28
, Issue.4
, pp. 693-705
-
-
Clayton, D.A.1
-
34
-
-
0034598918
-
Coupled leading-and lagging-strand synthesis of mammalian mitochondrial DNA
-
Holt I. J., Lorimer H. E., Jacobs H. T., Coupled leading-and lagging-strand synthesis of mammalian mitochondrial DNA Cell 2000 100 5 515 524
-
(2000)
Cell
, vol.100
, Issue.5
, pp. 515-524
-
-
Holt, I.J.1
Lorimer, H.E.2
Jacobs, H.T.3
-
35
-
-
20444428352
-
A bidirectional origin of replication maps to the major noncoding region of human mitochondrial DNA
-
Yasukawa T., Yang M.-Y., Jacobs H. T., Holt I. J., A bidirectional origin of replication maps to the major noncoding region of human mitochondrial DNA Molecular Cell 2005 18 6 651 662
-
(2005)
Molecular Cell
, vol.18
, Issue.6
, pp. 651-662
-
-
Yasukawa, T.1
Yang, M.-Y.2
Jacobs, H.T.3
Holt, I.J.4
-
36
-
-
33644635644
-
DNA polymerase in mitochondrial DNA replication and repair
-
Graziewicz M. A., Longley M. J., Copeland W. C., DNA polymerase in mitochondrial DNA replication and repair Chemical Reviews 2006 106 2 383 405
-
(2006)
Chemical Reviews
, vol.106
, Issue.2
, pp. 383-405
-
-
Graziewicz, M.A.1
Longley, M.J.2
Copeland, W.C.3
-
37
-
-
34548159301
-
Mammalian mitochondrial nucleoids: Organizing an independently minded genome
-
Holt I. J., He J., Mao C.-C., Mammalian mitochondrial nucleoids: organizing an independently minded genome Mitochondrion 2007 7 5 311 321
-
(2007)
Mitochondrion
, vol.7
, Issue.5
, pp. 311-321
-
-
Holt, I.J.1
He, J.2
Mao, C.-C.3
-
38
-
-
33745748154
-
Organization, dynamics and transmission of mitochondrial DNA: Focus on vertebrate nucleoids
-
Malka F., Lombs A., Rojo M., Organization, dynamics and transmission of mitochondrial DNA: focus on vertebrate nucleoids Biochimica et Biophysica Acta 2006 1763 5-6 463 472
-
(2006)
Biochimica et Biophysica Acta
, vol.1763
, Issue.56
, pp. 463-472
-
-
Malka, F.1
Lombs, A.2
Rojo, M.3
-
39
-
-
16844369889
-
Nuclear and mitochondrial DNA repair: Similar pathways?
-
Larsen N. B., Rasmussen M., Rasmussen L. J., Nuclear and mitochondrial DNA repair: similar pathways? Mitochondrion 2005 5 2 89 108
-
(2005)
Mitochondrion
, vol.5
, Issue.2
, pp. 89-108
-
-
Larsen, N.B.1
Rasmussen, M.2
Rasmussen, L.J.3
-
40
-
-
33644992894
-
Mitochondrial DNA maintenance and bioenergetics
-
Stuart J. A., Brown M. F., Mitochondrial DNA maintenance and bioenergetics Biochimica et Biophysica Acta 2006 1757 2 79 89
-
(2006)
Biochimica et Biophysica Acta
, vol.1757
, Issue.2
, pp. 79-89
-
-
Stuart, J.A.1
Brown, M.F.2
-
41
-
-
10344253295
-
Deoxyribonucleotides and disorders of mitochondrial DNA integrity
-
Saada A., Deoxyribonucleotides and disorders of mitochondrial DNA integrity DNA and Cell Biology 2004 23 12 797 806
-
(2004)
DNA and Cell Biology
, vol.23
, Issue.12
, pp. 797-806
-
-
Saada, A.1
-
42
-
-
23644436319
-
Disorders of nuclear-mitochondrial intergenomic signaling
-
Spinazzola A., Zeviani M., Disorders of nuclear-mitochondrial intergenomic signaling Gene 2005 354 1-2 162 168
-
(2005)
Gene
, vol.354
, Issue.12
, pp. 162-168
-
-
Spinazzola, A.1
Zeviani, M.2
-
43
-
-
49749141079
-
Approaches to finding the molecular basis of mitochondrial oxidative phosphorylation disorders
-
Kirby D. M., Thorburn D. R., Approaches to finding the molecular basis of mitochondrial oxidative phosphorylation disorders Twin Research and Human Genetics 2008 11 4 395 411
-
(2008)
Twin Research and Human Genetics
, vol.11
, Issue.4
, pp. 395-411
-
-
Kirby, D.M.1
Thorburn, D.R.2
-
44
-
-
33845744837
-
Initiation and beyond: Multiple functions of the human mitochondrial transcription machinery
-
Bonawitz N. D., Clayton D. A., Shadel G. S., Initiation and beyond: multiple functions of the human mitochondrial transcription machinery Molecular Cell 2006 24 6 813 825
-
(2006)
Molecular Cell
, vol.24
, Issue.6
, pp. 813-825
-
-
Bonawitz, N.D.1
Clayton, D.A.2
Shadel, G.S.3
-
46
-
-
65449130464
-
The MTERF family proteins: Mitochondrial transcription regulators and beyond
-
Roberti M., Polosa P. L., Bruni F., The MTERF family proteins: mitochondrial transcription regulators and beyond Biochimica et Biophysica Acta 2009 1787 5 303 311
-
(2009)
Biochimica et Biophysica Acta
, vol.1787
, Issue.5
, pp. 303-311
-
-
Roberti, M.1
Polosa, P.L.2
Bruni, F.3
-
47
-
-
36148945648
-
The mitochondrial transcription termination factor mTERF modulates replication pausing in human mitochondrial DNA
-
Hyvrinen A. K., Pohjoismki J. L. O., Reyes A., The mitochondrial transcription termination factor mTERF modulates replication pausing in human mitochondrial DNA Nucleic Acids Research 2007 35 19 6458 6474
-
(2007)
Nucleic Acids Research
, vol.35
, Issue.19
, pp. 6458-6474
-
-
Hyvrinen, A.K.1
Pohjoismki, J.L.O.2
Reyes, A.3
-
48
-
-
28044471185
-
A family of putative transcription termination factors shared amongst metazoans and plants
-
Linder T., Park C. B., Asin-Cayuela J., A family of putative transcription termination factors shared amongst metazoans and plants Current Genetics 2005 48 4 265 269
-
(2005)
Current Genetics
, vol.48
, Issue.4
, pp. 265-269
-
-
Linder, T.1
Park, C.B.2
Asin-Cayuela, J.3
-
49
-
-
27144452583
-
Cloning and functional analysis of human mTERFL encoding a novel mitochondrial transcription termination factor-like protein
-
Chen Y., Zhou G., Yu M., Cloning and functional analysis of human mTERFL encoding a novel mitochondrial transcription termination factor-like protein Biochemical and Biophysical Research Communications 2005 337 4 1112 1118
-
(2005)
Biochemical and Biophysical Research Communications
, vol.337
, Issue.4
, pp. 1112-1118
-
-
Chen, Y.1
Zhou, G.2
Yu, M.3
-
50
-
-
65449161792
-
MTERF2 is a nucleoid component in mammalian mitochondria
-
Pellegrini M., Asin-Cayuela J., Erdjument-Bromage H., Tempst P., Larsson N.-G., Gustafsson C. M., MTERF2 is a nucleoid component in mammalian mitochondria Biochimica et Biophysica Acta 2009 1787 5 296 302
-
(2009)
Biochimica et Biophysica Acta
, vol.1787
, Issue.5
, pp. 296-302
-
-
Pellegrini, M.1
Asin-Cayuela, J.2
Erdjument-Bromage, H.3
Tempst, P.4
Larsson, N.-G.5
Gustafsson, C.M.6
-
51
-
-
66049104206
-
MTERF2 regulates oxidative phosphorylation by modulating mtDNA transcription
-
Wenz T., Luca C., Torraco A., Moraes C. T., mTERF2 regulates oxidative phosphorylation by modulating mtDNA transcription Cell Metabolism 2009 9 6 499 511
-
(2009)
Cell Metabolism
, vol.9
, Issue.6
, pp. 499-511
-
-
Wenz, T.1
Luca, C.2
Torraco, A.3
Moraes, C.T.4
-
52
-
-
34447539564
-
MTERF3 is a negative regulator of mammalian mtDNA transcription
-
Park C. B., Asin-Cayuela J., Cmara Y., MTERF3 is a negative regulator of mammalian mtDNA transcription Cell 2007 130 2 273 285
-
(2007)
Cell
, vol.130
, Issue.2
, pp. 273-285
-
-
Park, C.B.1
Asin-Cayuela, J.2
Cmara, Y.3
-
53
-
-
33748962565
-
MTERF3, the most conserved member of the mTERF-family, is a modular factor involved in mitochondrial protein synthesis
-
Roberti M., Bruni F., Loguercio Polosa P., Manzari C., Gadaleta M. N., Cantatore P., MTERF3, the most conserved member of the mTERF-family, is a modular factor involved in mitochondrial protein synthesis Biochimica et Biophysica Acta 2006 1757 9-10 1199 1206
-
(2006)
Biochimica et Biophysica Acta
, vol.1757
, Issue.910
, pp. 1199-1206
-
-
Roberti, M.1
Bruni, F.2
Loguercio Polosa, P.3
Manzari, C.4
Gadaleta, M.N.5
Cantatore, P.6
-
54
-
-
77952507710
-
Initial characterization of MTERF4, a paralogue of MTERF1 (mTERF)
-
June, Stockholm, Sweden abstract no. 14
-
Cayuela A., Shi Y., Gustafsson C. M., Initial characterization of MTERF4, a paralogue of MTERF1 (mTERF) Proceedings of the 7th European Meeting on Mitochondrial Pathology (EUROMIT 08) June 2008 Stockholm, Sweden 20. abstract no. 14
-
(2008)
Proceedings of the 7th European Meeting on Mitochondrial Pathology (EUROMIT 08)
, pp. 20
-
-
Cayuela, A.1
Shi, Y.2
Gustafsson, C.M.3
-
55
-
-
0019444843
-
TRNA punctuation model of RNA processing in human mitochondria
-
Ojala D., Montoya J., Attardi G., tRNA punctuation model of RNA processing in human mitochondria Nature 1981 290 5806 470 474
-
(1981)
Nature
, vol.290
, Issue.5806
, pp. 470-474
-
-
Ojala, D.1
Montoya, J.2
Attardi, G.3
-
56
-
-
33748983242
-
Mitochondrial DNA transcription and diseases: Past, present and future
-
Montoya J., Lpez-Prez M. J., Ruiz-Pesini E., Mitochondrial DNA transcription and diseases: past, present and future Biochimica et Biophysica Acta 2006 1757 9-10 1179 1189
-
(2006)
Biochimica et Biophysica Acta
, vol.1757
, Issue.910
, pp. 1179-1189
-
-
Montoya, J.1
Lpez-Prez, M.J.2
Ruiz-Pesini, E.3
-
57
-
-
2442530075
-
Messenger RNA stability in mitochondria: Different means to an end
-
Gagliardi D., Stepien P. P., Temperley R. J., Lightowlers R. N., Chrzanowska-Lightowlers Z. M. A., Messenger RNA stability in mitochondria: different means to an end Trends in Genetics 2004 20 6 260 267
-
(2004)
Trends in Genetics
, vol.20
, Issue.6
, pp. 260-267
-
-
Gagliardi, D.1
Stepien, P.P.2
Temperley, R.J.3
Lightowlers, R.N.4
Chrzanowska-Lightowlers, Z.M.A.5
-
59
-
-
55249119103
-
Role of SUV3 helicase in maintaining mitochondrial homeostasis in human cells
-
Khidr L., Wu G., Davila A., Procaccio V., Wallace D., Lee W.-H., Role of SUV3 helicase in maintaining mitochondrial homeostasis in human cells Journal of Biological Chemistry 2008 283 40 27064 27073
-
(2008)
Journal of Biological Chemistry
, vol.283
, Issue.40
, pp. 27064-27073
-
-
Khidr, L.1
Wu, G.2
Davila, A.3
Procaccio, V.4
Wallace, D.5
Lee, W.-H.6
-
60
-
-
0026608057
-
MELAS mutation in mtDNA binding site for transcription termination factor causes defects in protein synthesis and in respiration but no change in levels of upstream and downstream mature transcripts
-
Chomyn A., Martinuzzi A., Yoneda M., MELAS mutation in mtDNA binding site for transcription termination factor causes defects in protein synthesis and in respiration but no change in levels of upstream and downstream mature transcripts Proceedings of the National Academy of Sciences of the United States of America 1992 89 10 4221 4225
-
(1992)
Proceedings of the National Academy of Sciences of the United States of America
, vol.89
, Issue.10
, pp. 4221-4225
-
-
Chomyn, A.1
Martinuzzi, A.2
Yoneda, M.3
-
61
-
-
0025845270
-
Impairment of mitochondrial transcription termination by a point mutation associated with the MELAS subgroup of mitochondrial encephalomyopathies
-
Hess J. F., Parisi M. A., Bennett J. L., Clayton D. A., Impairment of mitochondrial transcription termination by a point mutation associated with the MELAS subgroup of mitochondrial encephalomyopathies Nature 1991 351 6323 236 239
-
(1991)
Nature
, vol.351
, Issue.6323
, pp. 236-239
-
-
Hess, J.F.1
Parisi, M.A.2
Bennett, J.L.3
Clayton, D.A.4
-
62
-
-
33746559647
-
Mutation in TRMU related to transfer RNA modification modulates the phenotypic expression of the deafness-associated mitochondrial 12S ribosomal RNA mutations
-
Guan M.-X., Yan Q., Li X., Mutation in TRMU related to transfer RNA modification modulates the phenotypic expression of the deafness-associated mitochondrial 12S ribosomal RNA mutations American Journal of Human Genetics 2006 79 2 291 302
-
(2006)
American Journal of Human Genetics
, vol.79
, Issue.2
, pp. 291-302
-
-
Guan, M.-X.1
Yan, Q.2
Li, X.3
-
63
-
-
0032475958
-
Import into mitochondria, folding and retrograde movement of fumarase in yeast
-
Knox C., Sass E., Neupert W., Pines O., Import into mitochondria, folding and retrograde movement of fumarase in yeast Journal of Biological Chemistry 1998 273 40 25587 25593
-
(1998)
Journal of Biological Chemistry
, vol.273
, Issue.40
, pp. 25587-25593
-
-
Knox, C.1
Sass, E.2
Neupert, W.3
Pines, O.4
-
64
-
-
1642311878
-
Ribosomes specifically bind to mammalian mitochondria via protease-sensitive proteins on the outer membrane
-
MacKenzie J. A., Payne R. M., Ribosomes specifically bind to mammalian mitochondria via protease-sensitive proteins on the outer membrane Journal of Biological Chemistry 2004 279 11 9803 9810
-
(2004)
Journal of Biological Chemistry
, vol.279
, Issue.11
, pp. 9803-9810
-
-
MacKenzie, J.A.1
Payne, R.M.2
-
65
-
-
0027482105
-
Co-translational protein import into mitochondria: An alternative view
-
Verner K., Co-translational protein import into mitochondria: an alternative view Trends in Biochemical Sciences 1993 18 10 366 371
-
(1993)
Trends in Biochemical Sciences
, vol.18
, Issue.10
, pp. 366-371
-
-
Verner, K.1
-
66
-
-
0036200898
-
Genome-wide analysis of mRNAs targeted to yeast mitochondria
-
Marc P., Margeot A., Devaux F., Blugeon C., Corral-Debrinski M., Jacq C., Genome-wide analysis of mRNAs targeted to yeast mitochondria EMBO Reports 2002 3 2 159 164
-
(2002)
EMBO Reports
, vol.3
, Issue.2
, pp. 159-164
-
-
Marc, P.1
Margeot, A.2
Devaux, F.3
Blugeon, C.4
Corral-Debrinski, M.5
Jacq, C.6
-
67
-
-
4344581067
-
A co-translational model to explain the in vivo import of proteins into HeLa cell mitochondria
-
Mukhopadhyay A., Ni L., Weiner H., A co-translational model to explain the in vivo import of proteins into HeLa cell mitochondria Biochemical Journal 2004 382 1 385 392
-
(2004)
Biochemical Journal
, vol.382
, Issue.1
, pp. 385-392
-
-
Mukhopadhyay, A.1
Ni, L.2
Weiner, H.3
-
68
-
-
33846811281
-
Mitochondria-associated yeast mRNAs and the biogenesis of molecular complexes
-
Garcia M., Darzacq X., Delaveau T., Jourdren L., Singer R. H., Jacq C., Mitochondria-associated yeast mRNAs and the biogenesis of molecular complexes Molecular Biology of the Cell 2007 18 2 362 368
-
(2007)
Molecular Biology of the Cell
, vol.18
, Issue.2
, pp. 362-368
-
-
Garcia, M.1
Darzacq, X.2
Delaveau, T.3
Jourdren, L.4
Singer, R.H.5
Jacq, C.6
-
69
-
-
23644453428
-
Why are many mRNAs translated to the vicinity of mitochondria: A role in protein complex assembly?
-
Margeot A., Garcia M., Wang W., Tetaud E., di Rago J. P., Jacq C., Why are many mRNAs translated to the vicinity of mitochondria: a role in protein complex assembly? Gene 2005 354 1-2 64 71
-
(2005)
Gene
, vol.354
, Issue.12
, pp. 64-71
-
-
Margeot, A.1
Garcia, M.2
Wang, W.3
Tetaud, E.4
Di Rago, J.P.5
Jacq, C.6
-
70
-
-
3242705680
-
The functional organization of mitochondrial genomes in human cells
-
Iborra F. J., Kimura H., Cook P. R., The functional organization of mitochondrial genomes in human cells BMC Biology 2004 2, article 9
-
(2004)
BMC Biology
, vol.29
-
-
Iborra, F.J.1
Kimura, H.2
Cook, P.R.3
-
73
-
-
36949034917
-
Molecular genetics of a patient with Mohr-Tranebjaerg syndrome due to a new mutation in the DDP1 gene
-
Blesa J. R., Solano A., Briones P., Prieto-Ruiz J. A., Hernndez-Yago J., Coria F., Molecular genetics of a patient with Mohr-Tranebjaerg syndrome due to a new mutation in the DDP1 gene NeuroMolecular Medicine 2007 9 4 285 291
-
(2007)
NeuroMolecular Medicine
, vol.9
, Issue.4
, pp. 285-291
-
-
Blesa, J.R.1
Solano, A.2
Briones, P.3
Prieto-Ruiz, J.A.4
Hernndez-Yago, J.5
Coria, F.6
-
74
-
-
0036501592
-
Human deafness dystonia syndrome is caused by a defect in assembly of the DDP1/TIMM8a-TIMM13 complex
-
Roesch K., Curran S. P., Tranebjaerg L., Koehler C. M., Human deafness dystonia syndrome is caused by a defect in assembly of the DDP1/TIMM8a-TIMM13 complex Human Molecular Genetics 2002 11 5 477 486
-
(2002)
Human Molecular Genetics
, vol.11
, Issue.5
, pp. 477-486
-
-
Roesch, K.1
Curran, S.P.2
Tranebjaerg, L.3
Koehler, C.M.4
-
75
-
-
0042767609
-
Clinical and molecular findings in a patient with a novel mutation in the deafness-dystonia peptide (DDP1) gene
-
Binder J., Hofmann S., Kreisel S., Clinical and molecular findings in a patient with a novel mutation in the deafness-dystonia peptide (DDP1) gene Brain 2003 126 8 1814 1820
-
(2003)
Brain
, vol.126
, Issue.8
, pp. 1814-1820
-
-
Binder, J.1
Hofmann, S.2
Kreisel, S.3
-
76
-
-
33646427709
-
Mutation of DNAJC19, a human homologue of yeast inner mitochondrial membrane co-chaperones, causes DCMA syndrome, a novel autosomal recessive Barth syndrome-like condition
-
Davey K. M., Parboosingh J. S., McLeod D. R., Mutation of DNAJC19, a human homologue of yeast inner mitochondrial membrane co-chaperones, causes DCMA syndrome, a novel autosomal recessive Barth syndrome-like condition Journal of Medical Genetics 2006 43 5 385 393
-
(2006)
Journal of Medical Genetics
, vol.43
, Issue.5
, pp. 385-393
-
-
Davey, K.M.1
Parboosingh, J.S.2
McLeod, D.R.3
-
77
-
-
0141865519
-
Tim14, a novel key component of the import motor of the TIM23 protein translocase of mitochondria
-
Mokranjac D., Sichting M., Neupert W., Hell K., Tim14, a novel key component of the import motor of the TIM23 protein translocase of mitochondria EMBO Journal 2003 22 19 4945 4956
-
(2003)
EMBO Journal
, vol.22
, Issue.19
, pp. 4945-4956
-
-
Mokranjac, D.1
Sichting, M.2
Neupert, W.3
Hell, K.4
-
78
-
-
57849093113
-
Protein synthesis and assembly in mitochondrial disorders
-
Prez-Martnez X., Funes S., Camacho-Villasana Y., Marjavaara S., Tavares-Carren F., Shing-Vzquez M., Protein synthesis and assembly in mitochondrial disorders Current Topics in Medicinal Chemistry 2008 8 15 1335 1350
-
(2008)
Current Topics in Medicinal Chemistry
, vol.8
, Issue.15
, pp. 1335-1350
-
-
Prez-Martnez, X.1
Funes, S.2
Camacho-Villasana, Y.3
Marjavaara, S.4
Tavares-Carren, F.5
Shing-Vzquez, M.6
-
79
-
-
0026574207
-
Recent evidence for evolution of the genetic code
-
Osawa S., Jukes T. H., Watanabe K., Muto A., Recent evidence for evolution of the genetic code Microbiological Reviews 1992 56 1 229 264
-
(1992)
Microbiological Reviews
, vol.56
, Issue.1
, pp. 229-264
-
-
Osawa, S.1
Jukes, T.H.2
Watanabe, K.3
Muto, A.4
-
80
-
-
0019423857
-
Distinctive features of the 5 ′-terminal sequences of the human mitochondrial mRNAs
-
Montoya J., Ojala D., Attardi G., Distinctive features of the 5 ′-terminal sequences of the human mitochondrial mRNAs Nature 1981 290 5806 465 470
-
(1981)
Nature
, vol.290
, Issue.5806
, pp. 465-470
-
-
Montoya, J.1
Ojala, D.2
Attardi, G.3
-
81
-
-
0017802101
-
Failure to detect cap structures in mitochondrial DNA-coded poly(A)-containing RNA from HeLa cells
-
Grohmann K., Amalric F., Crews S., Attardi G., Failure to detect cap structures in mitochondrial DNA-coded poly(A)-containing RNA from HeLa cells Nucleic Acids Research 1978 5 3 637 651
-
(1978)
Nucleic Acids Research
, vol.5
, Issue.3
, pp. 637-651
-
-
Grohmann, K.1
Amalric, F.2
Crews, S.3
Attardi, G.4
-
82
-
-
0024542425
-
Interaction of bovine mitochondrial ribosomes with messenger RNA
-
Liao H.-X., Spremulli L. L., Interaction of bovine mitochondrial ribosomes with messenger RNA Journal of Biological Chemistry 1989 264 13 7518 7522
-
(1989)
Journal of Biological Chemistry
, vol.264
, Issue.13
, pp. 7518-7522
-
-
Liao, H.-X.1
Spremulli, L.L.2
-
83
-
-
14844340954
-
Initiation of protein synthesis in bacteria
-
Laursen B. S., Srensen H. P., Mortensen K. K., Sperling-Petersen H. U., Initiation of protein synthesis in bacteria Microbiology and Molecular Biology Reviews 2005 69 1 101 123
-
(2005)
Microbiology and Molecular Biology Reviews
, vol.69
, Issue.1
, pp. 101-123
-
-
Laursen, B.S.1
Srensen, H.P.2
Mortensen, K.K.3
Sperling-Petersen, H.U.4
-
84
-
-
27544482358
-
Protein synthesis in eukaryotes: The growing biological relevance of cap-independent translation initiation
-
Lpez-Lastra M., Rivas A., Barra M. I., Protein synthesis in eukaryotes: the growing biological relevance of cap-independent translation initiation Biological Research 2005 38 2-3 121 146
-
(2005)
Biological Research
, vol.38
, Issue.23
, pp. 121-146
-
-
Lpez-Lastra, M.1
Rivas, A.2
Barra, M.I.3
-
86
-
-
0021087964
-
Human mitochondrial genome and the evolution of methionine transfer ribonucleic acids
-
Mikelsaar R., Human mitochondrial genome and the evolution of methionine transfer ribonucleic acids Journal of Theoretical Biology 1983 105 2 221 232
-
(1983)
Journal of Theoretical Biology
, vol.105
, Issue.2
, pp. 221-232
-
-
Mikelsaar, R.1
-
87
-
-
25644436944
-
Translation initiation: Structures, mechanisms and evolution
-
Marintchev A., Wagner G., Translation initiation: structures, mechanisms and evolution Quarterly Reviews of Biophysics 2004 37 3-4 197 284
-
(2004)
Quarterly Reviews of Biophysics
, vol.37
, Issue.34
, pp. 197-284
-
-
Marintchev, A.1
Wagner, G.2
-
88
-
-
0035660343
-
Engaging the ribosome: Universal IFs of translation
-
Roll-Mecak A., Shin B.-S., Dever T. E., Burley S. K., Engaging the ribosome: universal IFs of translation Trends in Biochemical Sciences 2001 26 12 705 709
-
(2001)
Trends in Biochemical Sciences
, vol.26
, Issue.12
, pp. 705-709
-
-
Roll-Mecak, A.1
Shin, B.-S.2
Dever, T.E.3
Burley, S.K.4
-
89
-
-
0028959298
-
Cloning and sequence analysis of the human mitochondrial translational initiation factor 2 cDNA
-
Ma L., Spremulli L. L., Cloning and sequence analysis of the human mitochondrial translational initiation factor 2 cDNA Journal of Biological Chemistry 1995 270 4 1859 1865
-
(1995)
Journal of Biological Chemistry
, vol.270
, Issue.4
, pp. 1859-1865
-
-
Ma, L.1
Spremulli, L.L.2
-
90
-
-
0037144517
-
Identification of mammalian mitochondrial translational initiation factor 3 and examination of its role in initiation complex formation with natural mRNAs
-
Koc E. C., Spremulli L. L., Identification of mammalian mitochondrial translational initiation factor 3 and examination of its role in initiation complex formation with natural mRNAs Journal of Biological Chemistry 2002 277 38 35541 35549
-
(2002)
Journal of Biological Chemistry
, vol.277
, Issue.38
, pp. 35541-35549
-
-
Koc, E.C.1
Spremulli, L.L.2
-
91
-
-
4143062315
-
Initiation and elongation factors in mammalian mitochondrial protein biosynthesis
-
Spremulli L. L., Coursey A., Navratil T., Hunter S. E., Initiation and elongation factors in mammalian mitochondrial protein biosynthesis Progress in Nucleic Acid Research and Molecular Biology 2004 77 211 261
-
(2004)
Progress in Nucleic Acid Research and Molecular Biology
, vol.77
, pp. 211-261
-
-
Spremulli, L.L.1
Coursey, A.2
Navratil, T.3
Hunter, S.E.4
-
92
-
-
38649115355
-
A single mammalian mitochondrial translation initiation factor functionally replaces two bacterial factors
-
Gaur R., Grasso D., Datta P. P., A single mammalian mitochondrial translation initiation factor functionally replaces two bacterial factors Molecular Cell 2008 29 2 180 190
-
(2008)
Molecular Cell
, vol.29
, Issue.2
, pp. 180-190
-
-
Gaur, R.1
Grasso, D.2
Datta, P.P.3
-
93
-
-
0035177462
-
Identification and characterization of two novel human mitochondrial elongation factor genes, hEFG2 and hEFG1, phylogenetically conserved through evolution
-
Hammarsund M., Wilson W., Corcoran M., Identification and characterization of two novel human mitochondrial elongation factor genes, hEFG2 and hEFG1, phylogenetically conserved through evolution Human Genetics 2001 109 5 542 550
-
(2001)
Human Genetics
, vol.109
, Issue.5
, pp. 542-550
-
-
Hammarsund, M.1
Wilson, W.2
Corcoran, M.3
-
94
-
-
0030874533
-
The human mitochondrial elongation factor tu (EF-Tu) gene: CDNA sequence, genomic localization, genomic structure, and identification of a pseudogene
-
Ling M., Merante F., Chen H.-S., Duff C., Duncan A. M. V., Robinson B. H., The human mitochondrial elongation factor tu (EF-Tu) gene: CDNA sequence, genomic localization, genomic structure, and identification of a pseudogene Gene 1997 197 1-2 325 336
-
(1997)
Gene
, vol.197
, Issue.12
, pp. 325-336
-
-
Ling, M.1
Merante, F.2
Chen, H.-S.3
Duff, C.4
Duncan, A.M.V.5
Robinson, B.H.6
-
95
-
-
0029088252
-
Cloning and expression of mitochondrial translational elongation factor Ts from bovine and human liver
-
Xin H., Woriax V., Burkhart W., Spremulli L. L., Cloning and expression of mitochondrial translational elongation factor Ts from bovine and human liver Journal of Biological Chemistry 1995 270 29 17243 17249
-
(1995)
Journal of Biological Chemistry
, vol.270
, Issue.29
, pp. 17243-17249
-
-
Xin, H.1
Woriax, V.2
Burkhart, W.3
Spremulli, L.L.4
-
96
-
-
4444306660
-
Expression and characterization of isoform 1 of human mitochondrial elongation factor G
-
Bhargava K., Templeton P., Spremulli L. L., Expression and characterization of isoform 1 of human mitochondrial elongation factor G Protein Expression and Purification 2004 37 2 368 376
-
(2004)
Protein Expression and Purification
, vol.37
, Issue.2
, pp. 368-376
-
-
Bhargava, K.1
Templeton, P.2
Spremulli, L.L.3
-
97
-
-
0033529707
-
Functional characterization of the S. cerevisiae genome by gene deletion and parallel analysis
-
Winzeler E. A., Shoemaker D. D., Astromoff A., Functional characterization of the S. cerevisiae genome by gene deletion and parallel analysis Science 1999 285 5429 901 906
-
(1999)
Science
, vol.285
, Issue.5429
, pp. 901-906
-
-
Winzeler, E.A.1
Shoemaker, D.D.2
Astromoff, A.3
-
98
-
-
68949204220
-
EF-G2mt is an exclusive recycling factor in mammalian mitochondrial protein synthesis
-
Tsuboi M., Morita H., Nozaki Y., EF-G2mt is an exclusive recycling factor in mammalian mitochondrial protein synthesis Molecular Cell 2009 35 4 502 510
-
(2009)
Molecular Cell
, vol.35
, Issue.4
, pp. 502-510
-
-
Tsuboi, M.1
Morita, H.2
Nozaki, Y.3
-
99
-
-
0035137890
-
Endless possibilities: Translation termination and stop codon recognition
-
Bertram G., Innes S., Minella O., Richardson J. P., Stansfield I., Endless possibilities: translation termination and stop codon recognition Microbiology 2001 147 2 255 269 (Pubitemid 32156802)
-
(2001)
Microbiology
, vol.147
, Issue.2
, pp. 255-269
-
-
Bertram, G.1
Innes, S.2
Minella, O.3
Richardson, J.P.4
Stansfield, I.5
-
100
-
-
3943080710
-
The molecular mechanics of eukaryotic translation
-
Kapp L. D., Lorsch J. R., The molecular mechanics of eukaryotic translation Annual Review of Biochemistry 2004 73 657 704
-
(2004)
Annual Review of Biochemistry
, vol.73
, pp. 657-704
-
-
Kapp, L.D.1
Lorsch, J.R.2
-
101
-
-
42449108199
-
HMRF1L is a human mitochondrial translation release factor involved in the decoding of the termination codons UAA and UAG
-
Nozaki Y., Matsunaga N., Ishizawa T., Ueda T., Takeuchi N., HMRF1L is a human mitochondrial translation release factor involved in the decoding of the termination codons UAA and UAG Genes to Cells 2008 13 5 429 438
-
(2008)
Genes to Cells
, vol.13
, Issue.5
, pp. 429-438
-
-
Nozaki, Y.1
Matsunaga, N.2
Ishizawa, T.3
Ueda, T.4
Takeuchi, N.5
-
102
-
-
34548276891
-
MtRF1a is a human mitochondrial translation release factor decoding the major termination codons UAA and UAG
-
Soleimanpour-Lichaei H. R., Khl I., Gaisne M., mtRF1a is a human mitochondrial translation release factor decoding the major termination codons UAA and UAG Molecular Cell 2007 27 5 745 757
-
(2007)
Molecular Cell
, vol.27
, Issue.5
, pp. 745-757
-
-
Soleimanpour-Lichaei, H.R.1
Khl, I.2
Gaisne, M.3
-
103
-
-
0032570020
-
Identification and cloning of human mitochondrial translational release factor 1 and the ribosome recycling factor
-
Zhang Y., Spremulli L. L., Identification and cloning of human mitochondrial translational release factor 1 and the ribosome recycling factor Biochimica et Biophysica Acta 1998 1443 1-2 245 250
-
(1998)
Biochimica et Biophysica Acta
, vol.1443
, Issue.12
, pp. 245-250
-
-
Zhang, Y.1
Spremulli, L.L.2
-
104
-
-
54549116197
-
The human mitochondrial ribosome recycling factor is essential for cell viability
-
Rorbach J., Richter R., Wessels H. J., The human mitochondrial ribosome recycling factor is essential for cell viability Nucleic Acids Research 2008 36 18 5787 5799
-
(2008)
Nucleic Acids Research
, vol.36
, Issue.18
, pp. 5787-5799
-
-
Rorbach, J.1
Richter, R.2
Wessels, H.J.3
-
105
-
-
0019837699
-
Sequence and gene organization of mouse mitochondrial DNA
-
Bibb M. J., Van Etten R. A., Wright C. T., Walberg M. W., Clayton D. A., Sequence and gene organization of mouse mitochondrial DNA Cell 1981 26 2 167 180
-
(1981)
Cell
, vol.26
, Issue.2
, pp. 167-180
-
-
Bibb, M.J.1
Van Etten, R.A.2
Wright, C.T.3
Walberg, M.W.4
Clayton, D.A.5
-
106
-
-
0024352214
-
The complete nucleotide sequence of the Rattus norvegicus mitochondrial genome: Cryptic signals revealed by comparative analysis between vertebrates
-
Gadaleta G., Pepe G., De Candia G., Quagliariello C., Sbisa E., Saccone C., The complete nucleotide sequence of the Rattus norvegicus mitochondrial genome: cryptic signals revealed by comparative analysis between vertebrates Journal of Molecular Evolution 1989 28 6 497 516
-
(1989)
Journal of Molecular Evolution
, vol.28
, Issue.6
, pp. 497-516
-
-
Gadaleta, G.1
Pepe, G.2
De Candia, G.3
Quagliariello, C.4
Sbisa, E.5
Saccone, C.6
-
107
-
-
34547894695
-
Reconstructing the evolution of the mitochondrial ribosomal proteome
-
Smits P., Smeitink J. A. M., van den Heuvel L. P., Huynen M. A., Ettema T. J. G., Reconstructing the evolution of the mitochondrial ribosomal proteome Nucleic Acids Research 2007 35 14 4686 4703
-
(2007)
Nucleic Acids Research
, vol.35
, Issue.14
, pp. 4686-4703
-
-
Smits, P.1
Smeitink, J.A.M.2
Van Den Heuvel, L.P.3
Huynen, M.A.4
Ettema, T.J.G.5
-
108
-
-
0344012611
-
Properties of human mitochondrial ribosomes
-
OBrien T. W., Properties of human mitochondrial ribosomes IUBMB Life 2003 55 9 505 513
-
(2003)
IUBMB Life
, vol.55
, Issue.9
, pp. 505-513
-
-
Obrien, T.W.1
-
109
-
-
0141953259
-
Structure of the mammalian mitochondrial ribosome reveals an expanded functional role for its component proteins
-
Sharma M. R., Koc E. C., Datta P. P., Booth T. M., Spremulli L. L., Agrawal R. K., Structure of the mammalian mitochondrial ribosome reveals an expanded functional role for its component proteins Cell 2003 115 1 97 108
-
(2003)
Cell
, vol.115
, Issue.1
, pp. 97-108
-
-
Sharma, M.R.1
Koc, E.C.2
Datta, P.P.3
Booth, T.M.4
Spremulli, L.L.5
Agrawal, R.K.6
-
110
-
-
0033735040
-
Search for characteristic structural features of mammalian mitochondrial tRNAs
-
Helm M., Brul H., Friede D., Gieg R., Ptz D., Florentz C., Search for characteristic structural features of mammalian mitochondrial tRNAs RNA 2000 6 10 1356 1379
-
(2000)
RNA
, vol.6
, Issue.10
, pp. 1356-1379
-
-
Helm, M.1
Brul, H.2
Friede, D.3
Gieg, R.4
Ptz, D.5
Florentz, C.6
-
111
-
-
32644447756
-
Post-transcriptional nucleotide modification and alternative folding of RNA
-
Helm M., Post-transcriptional nucleotide modification and alternative folding of RNA Nucleic Acids Research 2006 34 2 721 733
-
(2006)
Nucleic Acids Research
, vol.34
, Issue.2
, pp. 721-733
-
-
Helm, M.1
-
112
-
-
0032867610
-
A Watson-Crick base-pair-disrupting methyl group (m1A9) is sufficient for cloverleaf folding of human mitochondrial tRNA(Lys)
-
Helm M., Gieg R., Florentz C., A Watson-Crick base-pair-disrupting methyl group (m1A9) is sufficient for cloverleaf folding of human mitochondrial tRNA(Lys) Biochemistry 1999 38 40 13338 13346
-
(1999)
Biochemistry
, vol.38
, Issue.40
, pp. 13338-13346
-
-
Helm, M.1
Gieg, R.2
Florentz, C.3
-
113
-
-
25444436228
-
Human mitochondrial diseases associated with tRNA wobble modification deficiency
-
Kirino Y., Suzuki T., Human mitochondrial diseases associated with tRNA wobble modification deficiency RNA Biology 2005 2 2 41 44 (Pubitemid 41359797)
-
(2005)
RNA Biology
, vol.2
, Issue.2
, pp. 41-44
-
-
Kirino, Y.1
Suzuki, T.2
-
114
-
-
3643114575
-
Universal rules and idiosyncratic features in tRNA identity
-
Gieg R., Sissler M., Florentz C., Universal rules and idiosyncratic features in tRNA identity Nucleic Acids Research 1998 26 22 5017 5035
-
(1998)
Nucleic Acids Research
, vol.26
, Issue.22
, pp. 5017-5035
-
-
Gieg, R.1
Sissler, M.2
Florentz, C.3
-
115
-
-
15444367104
-
Toward the full set of human mitochondrial aminoacyl-tRNA synthetases: Characterization of AspRS and TyrRS
-
Bonnefond L., Fender A., Rudinger-Thirion J., Gieg R., Florentz C., Sissler M., Toward the full set of human mitochondrial aminoacyl-tRNA synthetases: characterization of AspRS and TyrRS Biochemistry 2005 44 12 4805 4816
-
(2005)
Biochemistry
, vol.44
, Issue.12
, pp. 4805-4816
-
-
Bonnefond, L.1
Fender, A.2
Rudinger-Thirion, J.3
Gieg, R.4
Florentz, C.5
Sissler, M.6
-
116
-
-
67349234111
-
Pentatricopeptide repeat domain protein 3 associates with the mitochondrial small ribosomal subunit and regulates translation
-
Davies S. M. K., Rackham O., Shearwood A.-M. J., Pentatricopeptide repeat domain protein 3 associates with the mitochondrial small ribosomal subunit and regulates translation FEBS Letters 2009 583 12 1853 1858
-
(2009)
FEBS Letters
, vol.583
, Issue.12
, pp. 1853-1858
-
-
Davies, S.M.K.1
Rackham, O.2
Shearwood, A.-M.J.3
-
117
-
-
0037154986
-
Ribosome structure and the mechanism of translation
-
Ramakrishnan V., Ribosome structure and the mechanism of translation Cell 2002 108 4 557 572
-
(2002)
Cell
, vol.108
, Issue.4
, pp. 557-572
-
-
Ramakrishnan, V.1
-
118
-
-
42449139620
-
Lack of secondary structure characterizes the 5 ′ ends of mammalian mitochondrial mRNAs
-
Jones C. N., Wilkinson K. A., Hung K. T., Weeks K. M., Spremulli L. L., Lack of secondary structure characterizes the 5 ′ ends of mammalian mitochondrial mRNAs RNA 2008 14 5 862 871
-
(2008)
RNA
, vol.14
, Issue.5
, pp. 862-871
-
-
Jones, C.N.1
Wilkinson, K.A.2
Hung, K.T.3
Weeks, K.M.4
Spremulli, L.L.5
-
119
-
-
65249170067
-
Evidence for an active role of IF3mt in the initiation of translation in mammalian mitochondria
-
Christian B. E., Spremulli L. L., Evidence for an active role of IF3mt in the initiation of translation in mammalian mitochondria Biochemistry 2009 48 15 3269 3278
-
(2009)
Biochemistry
, vol.48
, Issue.15
, pp. 3269-3278
-
-
Christian, B.E.1
Spremulli, L.L.2
-
120
-
-
31144476909
-
Role of the N-and C-terminal extensions on the activity of mammalian mitochondrial translational initiation factor 3
-
Bhargava K., Spremulli L. L., Role of the N-and C-terminal extensions on the activity of mammalian mitochondrial translational initiation factor 3 Nucleic Acids Research 2005 33 22 7011 7018
-
(2005)
Nucleic Acids Research
, vol.33
, Issue.22
, pp. 7011-7018
-
-
Bhargava, K.1
Spremulli, L.L.2
-
121
-
-
0025076337
-
Identification and initial characterization of translational initiation factor 2 from bovine mitochondria
-
Liao H.-X., Spremulli L. L., Identification and initial characterization of translational initiation factor 2 from bovine mitochondria Journal of Biological Chemistry 1990 265 23 13618 13622
-
(1990)
Journal of Biological Chemistry
, vol.265
, Issue.23
, pp. 13618-13622
-
-
Liao, H.-X.1
Spremulli, L.L.2
-
122
-
-
0029913846
-
Expression, purification, and mechanistic studies of bovine mitochondrial translational initiation factor 2
-
Ma J., Spremulli L. L., Expression, purification, and mechanistic studies of bovine mitochondrial translational initiation factor 2 Journal of Biological Chemistry 1996 271 10 5805 5811
-
(1996)
Journal of Biological Chemistry
, vol.271
, Issue.10
, pp. 5805-5811
-
-
Ma, J.1
Spremulli, L.L.2
-
123
-
-
39149137835
-
The interaction of mammalian mitochondrial translational initiation factor 3 with ribosomes: Evolution of terminal extensions in IF3mt
-
Haque Md. E., Grasso D., Spremulli L. L., The interaction of mammalian mitochondrial translational initiation factor 3 with ribosomes: evolution of terminal extensions in IF3mt Nucleic Acids Research 2008 36 2 589 597
-
(2008)
Nucleic Acids Research
, vol.36
, Issue.2
, pp. 589-597
-
-
Haque, Md.E.1
Grasso, D.2
Spremulli, L.L.3
-
124
-
-
0034617088
-
Interaction of mitochondrial elongation factor Tu with aminoacyl-tRNA and elongation factor Ts
-
Cai Y.-C., Bullard J. M., Thompson N. L., Spremulli L. L., Interaction of mitochondrial elongation factor Tu with aminoacyl-tRNA and elongation factor Ts Journal of Biological Chemistry 2000 275 27 20308 20314
-
(2000)
Journal of Biological Chemistry
, vol.275
, Issue.27
, pp. 20308-20314
-
-
Cai, Y.-C.1
Bullard, J.M.2
Thompson, N.L.3
Spremulli, L.L.4
-
125
-
-
0031002606
-
Mechanistic studies of the translational elongation cycle in mammalian mitochondria
-
Woriax V. L., Bullard J. M., Ma L., Yokogawa T., Spremulli L. L., Mechanistic studies of the translational elongation cycle in mammalian mitochondria Biochimica et Biophysica Acta 1997 1352 1 91 101
-
(1997)
Biochimica et Biophysica Acta
, vol.1352
, Issue.1
, pp. 91-101
-
-
Woriax, V.L.1
Bullard, J.M.2
Ma, L.3
Yokogawa, T.4
Spremulli, L.L.5
-
126
-
-
56049087303
-
The A3243G tRNALeu(UUR) MELAS mutation causes amino acid misincorporation and a combined respiratory chain assembly defect partially suppressed by overexpression of EFTu and EFG2
-
Sasarman F., Antonicka H., Shoubridge E. A., The A3243G tRNALeu(UUR) MELAS mutation causes amino acid misincorporation and a combined respiratory chain assembly defect partially suppressed by overexpression of EFTu and EFG2 Human Molecular Genetics 2008 17 23 3697 3707
-
(2008)
Human Molecular Genetics
, vol.17
, Issue.23
, pp. 3697-3707
-
-
Sasarman, F.1
Antonicka, H.2
Shoubridge, E.A.3
-
127
-
-
42949101660
-
Aminoacyl-tRNA surveillance by EF-Tu in mammalian mitochondria
-
Nagao A., Suzuki T., Suzuki T., Aminoacyl-tRNA surveillance by EF-Tu in mammalian mitochondria Nucleic Acids Symposium Series 2007 51 41 42
-
(2007)
Nucleic Acids Symposium Series
, Issue.51
, pp. 41-42
-
-
Nagao, A.1
Suzuki, T.2
Suzuki, T.3
-
128
-
-
0036382768
-
Modeling a minimal ribosome based on comparative sequence analysis
-
Mears J. A., Cannone J. J., Stagg S. M., Gutell R. R., Agrawal R. K., Harvey S. C., Modeling a minimal ribosome based on comparative sequence analysis Journal of Molecular Biology 2002 321 2 215 234
-
(2002)
Journal of Molecular Biology
, vol.321
, Issue.2
, pp. 215-234
-
-
Mears, J.A.1
Cannone, J.J.2
Stagg, S.M.3
Gutell, R.R.4
Agrawal, R.K.5
Harvey, S.C.6
-
129
-
-
33645106536
-
A structural model for the large subunit of the mammalian mitochondrial ribosome
-
Mears J. A., Sharma M. R., Gutell R. R., A structural model for the large subunit of the mammalian mitochondrial ribosome Journal of Molecular Biology 2006 358 1 193 212
-
(2006)
Journal of Molecular Biology
, vol.358
, Issue.1
, pp. 193-212
-
-
Mears, J.A.1
Sharma, M.R.2
Gutell, R.R.3
-
130
-
-
31544450549
-
Regulation of mitochondrial translation in yeast
-
Towpik J., Regulation of mitochondrial translation in yeast Cellular and Molecular Biology Letters 2005 10 4 571 594
-
(2005)
Cellular and Molecular Biology Letters
, vol.10
, Issue.4
, pp. 571-594
-
-
Towpik, J.1
-
131
-
-
0347574101
-
Coupling of mitochondrial translation with the formation of respiratory complexes in yeast mitochondria
-
Chacinska A., Boguta M., Coupling of mitochondrial translation with the formation of respiratory complexes in yeast mitochondria Acta Biochimica Polonica 2000 47 4 973 991
-
(2000)
Acta Biochimica Polonica
, vol.47
, Issue.4
, pp. 973-991
-
-
Chacinska, A.1
Boguta, M.2
-
132
-
-
33846573772
-
The Saccharomyces cerevisiae ATP22 gene codes for the mitochondrial ATPase subunit 6-specific translation factor
-
Zeng X., Hourset A., Tzagoloff A., The Saccharomyces cerevisiae ATP22 gene codes for the mitochondrial ATPase subunit 6-specific translation factor Genetics 2007 175 1 55 63
-
(2007)
Genetics
, vol.175
, Issue.1
, pp. 55-63
-
-
Zeng, X.1
Hourset, A.2
Tzagoloff, A.3
-
133
-
-
0037345834
-
RNA-binding proteins of mammalian mitochondria
-
Koc E. C., Spremulli L. L., RNA-binding proteins of mammalian mitochondria Mitochondrion 2003 2 4 277 291
-
(2003)
Mitochondrion
, vol.2
, Issue.4
, pp. 277-291
-
-
Koc, E.C.1
Spremulli, L.L.2
-
134
-
-
67649833762
-
Mutation in TACO1, encoding a translational activator of COX I, results in cytochrome c oxidase deficiency and late-onset Leigh syndrome
-
Weraarpachai W., Antonicka H., Sasarman F., Mutation in TACO1, encoding a translational activator of COX I, results in cytochrome c oxidase deficiency and late-onset Leigh syndrome Nature Genetics 2009 41 7 833 837
-
(2009)
Nature Genetics
, vol.41
, Issue.7
, pp. 833-837
-
-
Weraarpachai, W.1
Antonicka, H.2
Sasarman, F.3
-
135
-
-
0035896652
-
Nam1p, a protein involved in RNA processing and translation, is coupled to transcription through an interaction with yeast mitochondrial RNA polymerase
-
Rodeheffer M. S., Boone B. E., Bryan A. C., Shadel G. S., Nam1p, a protein involved in RNA processing and translation, is coupled to transcription through an interaction with yeast mitochondrial RNA polymerase Journal of Biological Chemistry 2001 276 11 8616 8622
-
(2001)
Journal of Biological Chemistry
, vol.276
, Issue.11
, pp. 8616-8622
-
-
Rodeheffer, M.S.1
Boone, B.E.2
Bryan, A.C.3
Shadel, G.S.4
-
137
-
-
0028984035
-
The product of the nuclear gene PET309 is required for translation of mature mRNA and stability or production of intron-containing RNAs derived from the mitochondrial COX1 locus of Saccharomyces cerevisiae
-
Manthey G. M., McEwen J. E., The product of the nuclear gene PET309 is required for translation of mature mRNA and stability or production of intron-containing RNAs derived from the mitochondrial COX1 locus of Saccharomyces cerevisiae EMBO Journal 1995 14 16 4031 4043
-
(1995)
EMBO Journal
, vol.14
, Issue.16
, pp. 4031-4043
-
-
Manthey, G.M.1
McEwen, J.E.2
-
138
-
-
4344595430
-
The role of the LRPPRC (leucine-rich pentatricopeptide repeal cassette) gene in cytochrome oxidase assembly: Mutation causes lowered levels of COX (cytochrome c oxidase) i and COX III mRNA
-
Xu F., Morin C., Mitchell G., Ackerley C., Robinson B. H., The role of the LRPPRC (leucine-rich pentatricopeptide repeal cassette) gene in cytochrome oxidase assembly: mutation causes lowered levels of COX (cytochrome c oxidase) I and COX III mRNA Biochemical Journal 2004 382 1 331 336
-
(2004)
Biochemical Journal
, vol.382
, Issue.1
, pp. 331-336
-
-
Xu, F.1
Morin, C.2
Mitchell, G.3
Ackerley, C.4
Robinson, B.H.5
-
139
-
-
0037238395
-
Interactions among COX1, COX2, and COX3 mRNA-specific translational activator proteins on the inner surface of the mitochondrial inner membrane of Saccharomyces cerevisiae
-
Naithani S., Saracco S. A., Butler C. A., Fox T. D., Interactions among COX1, COX2, and COX3 mRNA-specific translational activator proteins on the inner surface of the mitochondrial inner membrane of Saccharomyces cerevisiae Molecular Biology of the Cell 2003 14 1 324 333
-
(2003)
Molecular Biology of the Cell
, vol.14
, Issue.1
, pp. 324-333
-
-
Naithani, S.1
Saracco, S.A.2
Butler, C.A.3
Fox, T.D.4
-
140
-
-
0028334071
-
The NAM1 protein (NAM1p), which is selectively required for cox1, cytb and atp6 transcript processing/stabilisation, is located in the yeast mitochondrial matrix
-
Wallis M. G., Groudinsky O., Slonimski P. P., Dujardin G., The NAM1 protein (NAM1p), which is selectively required for cox1, cytb and atp6 transcript processing/stabilisation, is located in the yeast mitochondrial matrix European Journal of Biochemistry 1994 222 1 27 32
-
(1994)
European Journal of Biochemistry
, vol.222
, Issue.1
, pp. 27-32
-
-
Wallis, M.G.1
Groudinsky, O.2
Slonimski, P.P.3
Dujardin, G.4
-
141
-
-
4444268726
-
Coupling the mitochondrial transcription machinery to human disease
-
Shadel G. S., Coupling the mitochondrial transcription machinery to human disease Trends in Genetics 2004 20 10 513 519
-
(2004)
Trends in Genetics
, vol.20
, Issue.10
, pp. 513-519
-
-
Shadel, G.S.1
-
142
-
-
0037774459
-
LRP130, a pentatricopeptide motif protein with a noncanonical RNA-binding domain, is bound in vivo to mitochondrial and nuclear RNAs
-
Mili S., Piol-Roma S., LRP130, a pentatricopeptide motif protein with a noncanonical RNA-binding domain, is bound in vivo to mitochondrial and nuclear RNAs Molecular and Cellular Biology 2003 23 14 4972 4982
-
(2003)
Molecular and Cellular Biology
, vol.23
, Issue.14
, pp. 4972-4982
-
-
Mili, S.1
Piol-Roma, S.2
-
143
-
-
26844484821
-
The m-AAA protease defective in hereditary spastic paraplegia controls ribosome assembly in mitochondria
-
Nolden M., Ehses S., Koppen M., Bernacchia A., Rugarli E. I., Langer T., The m-AAA protease defective in hereditary spastic paraplegia controls ribosome assembly in mitochondria Cell 2005 123 2 277 289
-
(2005)
Cell
, vol.123
, Issue.2
, pp. 277-289
-
-
Nolden, M.1
Ehses, S.2
Koppen, M.3
Bernacchia, A.4
Rugarli, E.I.5
Langer, T.6
-
144
-
-
0032511186
-
Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease
-
Casari G., De Fusco M., Ciarmatori S., Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease Cell 1998 93 6 973 983
-
(1998)
Cell
, vol.93
, Issue.6
, pp. 973-983
-
-
Casari, G.1
De Fusco, M.2
Ciarmatori, S.3
-
145
-
-
44849128599
-
Expression and maintenance of mitochondrial DNA: New insights into human disease pathology
-
Shadel G. S., Expression and maintenance of mitochondrial DNA: new insights into human disease pathology American Journal of Pathology 2008 172 6 1445 1456
-
(2008)
American Journal of Pathology
, vol.172
, Issue.6
, pp. 1445-1456
-
-
Shadel, G.S.1
-
146
-
-
34547621748
-
Relative abundance of the human mitochondrial transcription system and distinct roles for h-mtTFB1 and h-mtTFB2 in mitochondrial biogenesis and gene expression
-
Cotney J., Wang Z., Shadel G. S., Relative abundance of the human mitochondrial transcription system and distinct roles for h-mtTFB1 and h-mtTFB2 in mitochondrial biogenesis and gene expression Nucleic Acids Research 2007 35 12 4042 4054
-
(2007)
Nucleic Acids Research
, vol.35
, Issue.12
, pp. 4042-4054
-
-
Cotney, J.1
Wang, Z.2
Shadel, G.S.3
-
147
-
-
1942425120
-
Human mitochondrial transcription factor B1 as a modifier gene for hearing loss associated with the mitochondrial A1555G mutation
-
Bykhovskaya Y., Mengesha E., Wang D., Human mitochondrial transcription factor B1 as a modifier gene for hearing loss associated with the mitochondrial A1555G mutation Molecular Genetics and Metabolism 2004 82 1 27 32
-
(2004)
Molecular Genetics and Metabolism
, vol.82
, Issue.1
, pp. 27-32
-
-
Bykhovskaya, Y.1
Mengesha, E.2
Wang, D.3
-
148
-
-
63449105579
-
Methylation of 12S rRNA is necessary for in vivo stability of the small subunit of the mammalian mitochondrial ribosome
-
Metodiev M. D., Lesko N., Park C. B., Methylation of 12S rRNA is necessary for in vivo stability of the small subunit of the mammalian mitochondrial ribosome Cell Metabolism 2009 9 4 386 397
-
(2009)
Cell Metabolism
, vol.9
, Issue.4
, pp. 386-397
-
-
Metodiev, M.D.1
Lesko, N.2
Park, C.B.3
-
149
-
-
42049114034
-
Transcriptional paradigms in mammalian mitochondrial biogenesis and function
-
Scarpulla R. C., Transcriptional paradigms in mammalian mitochondrial biogenesis and function Physiological Reviews 2008 88 2 611 638
-
(2008)
Physiological Reviews
, vol.88
, Issue.2
, pp. 611-638
-
-
Scarpulla, R.C.1
-
150
-
-
68749094302
-
CAMP response element-binding protein (CREB) is imported into mitochondria and promotes protein synthesis
-
De Rasmo D., Signorile A., Roca E., Papa S., CAMP response element-binding protein (CREB) is imported into mitochondria and promotes protein synthesis FEBS Journal 2009 276 16 4325 4333
-
(2009)
FEBS Journal
, vol.276
, Issue.16
, pp. 4325-4333
-
-
De Rasmo, D.1
Signorile, A.2
Roca, E.3
Papa, S.4
-
151
-
-
33749150990
-
RNase L: Its biological roles and regulation
-
Liang S.-L., Quirk D., Zhou A., RNase L: its biological roles and regulation IUBMB Life 2006 58 9 508 514
-
(2006)
IUBMB Life
, vol.58
, Issue.9
, pp. 508-514
-
-
Liang, S.-L.1
Quirk, D.2
Zhou, A.3
-
152
-
-
34447636435
-
Regulation of mitochondrial mRNA stability by RNase L is translation-dependent and controls IFN-induced apoptosis
-
Le Roy F., Silhol M., Salehzada T., Bisbal C., Regulation of mitochondrial mRNA stability by RNase L is translation-dependent and controls IFN-induced apoptosis Cell Death and Differentiation 2007 14 8 1406 1413
-
(2007)
Cell Death and Differentiation
, vol.14
, Issue.8
, pp. 1406-1413
-
-
Le Roy, F.1
Silhol, M.2
Salehzada, T.3
Bisbal, C.4
-
153
-
-
33947518803
-
Chaperone properties of mammalian mitochondrial translation elongation factor Tu
-
Suzuki H., Ueda T., Taguchi H., Takeuchi N., Chaperone properties of mammalian mitochondrial translation elongation factor Tu Journal of Biological Chemistry 2007 282 6 4076 4084
-
(2007)
Journal of Biological Chemistry
, vol.282
, Issue.6
, pp. 4076-4084
-
-
Suzuki, H.1
Ueda, T.2
Taguchi, H.3
Takeuchi, N.4
-
154
-
-
17644372436
-
Peptidase activity of the Escherichia coli Hsp31 chaperone
-
Malki A., Caldas T., Abdallah J., Peptidase activity of the Escherichia coli Hsp31 chaperone Journal of Biological Chemistry 2005 280 15 14420 14426
-
(2005)
Journal of Biological Chemistry
, vol.280
, Issue.15
, pp. 14420-14426
-
-
Malki, A.1
Caldas, T.2
Abdallah, J.3
-
155
-
-
34250376484
-
Human mitochondrial ribosomal protein MRPL12 interacts directly with mitochondrial RNA polymerase to modulate mitochondrial gene expression
-
Wang Z., Cotney J., Shadel G. S., Human mitochondrial ribosomal protein MRPL12 interacts directly with mitochondrial RNA polymerase to modulate mitochondrial gene expression Journal of Biological Chemistry 2007 282 17 12610 12618
-
(2007)
Journal of Biological Chemistry
, vol.282
, Issue.17
, pp. 12610-12618
-
-
Wang, Z.1
Cotney, J.2
Shadel, G.S.3
-
156
-
-
0035831128
-
A new face on apoptosis: Death-associated protein 3 and PDCD9 are mitochondrial ribosomal proteins
-
Koc E. C., Ranasinghe A., Burkhart W., A new face on apoptosis: death-associated protein 3 and PDCD9 are mitochondrial ribosomal proteins FEBS Letters 2001 492 1-2 166 170
-
(2001)
FEBS Letters
, vol.492
, Issue.12
, pp. 166-170
-
-
Koc, E.C.1
Ranasinghe, A.2
Burkhart, W.3
-
157
-
-
0037009130
-
Molecular chaperones as essential mediators of mitochondrial biogenesis
-
Voos W., Rttgers K., Molecular chaperones as essential mediators of mitochondrial biogenesis Biochimica et Biophysica Acta 2002 1592 1 51 62
-
(2002)
Biochimica et Biophysica Acta
, vol.1592
, Issue.1
, pp. 51-62
-
-
Voos, W.1
Rttgers, K.2
-
158
-
-
34250369119
-
Protein degradation within mitochondria: Versatile activities of AAA proteases and other peptidases
-
Koppen M., Langer T., Protein degradation within mitochondria: versatile activities of AAA proteases and other peptidases Critical Reviews in Biochemistry and Molecular Biology 2007 42 3 221 242
-
(2007)
Critical Reviews in Biochemistry and Molecular Biology
, vol.42
, Issue.3
, pp. 221-242
-
-
Koppen, M.1
Langer, T.2
-
159
-
-
0036161635
-
The mitochondrial PHB complex: Roles in mitochondrial respiratory complex assembly, ageing and degenerative disease
-
Nijtmans L. G. J., Artal S. M., Grivell L. A., Coates P. J., The mitochondrial PHB complex: roles in mitochondrial respiratory complex assembly, ageing and degenerative disease Cellular and Molecular Life Sciences 2002 59 1 143 155
-
(2002)
Cellular and Molecular Life Sciences
, vol.59
, Issue.1
, pp. 143-155
-
-
Nijtmans, L.G.J.1
Artal, S.M.2
Grivell, L.A.3
Coates, P.J.4
-
161
-
-
0034703062
-
Interaction of mammalian mitochondrial ribosomes with the inner membrane
-
Liu M., Spremulli L., Interaction of mammalian mitochondrial ribosomes with the inner membrane Journal of Biological Chemistry 2000 275 38 29400 29406
-
(2000)
Journal of Biological Chemistry
, vol.275
, Issue.38
, pp. 29400-29406
-
-
Liu, M.1
Spremulli, L.2
-
162
-
-
0032478139
-
Oxa1p, an essential component of the N-tail protein export machinery in mitochondria
-
Hell K., Herrmann J. M., Pratje E., Neupert W., Stuart R. A., Oxa1p, an essential component of the N-tail protein export machinery in mitochondria Proceedings of the National Academy of Sciences of the United States of America 1998 95 5 2250 2255
-
(1998)
Proceedings of the National Academy of Sciences of the United States of America
, vol.95
, Issue.5
, pp. 2250-2255
-
-
Hell, K.1
Herrmann, J.M.2
Pratje, E.3
Neupert, W.4
Stuart, R.A.5
-
163
-
-
0035868763
-
Oxa1p acts as a general membrane insertion machinery for proteins encoded by mitochondrial DNA
-
Hell K., Neupert W., Stuart R. A., Oxa1p acts as a general membrane insertion machinery for proteins encoded by mitochondrial DNA EMBO Journal 2001 20 6 1281 1288
-
(2001)
EMBO Journal
, vol.20
, Issue.6
, pp. 1281-1288
-
-
Hell, K.1
Neupert, W.2
Stuart, R.A.3
-
164
-
-
33646138730
-
Mba1, a membrane-associated ribosome receptor in mitochondria
-
Ott M., Prestele M., Bauerschmitt H., Funes S., Bonnefoy N., Herrmann J. M., Mba1, a membrane-associated ribosome receptor in mitochondria EMBO Journal 2006 25 8 1603 1610
-
(2006)
EMBO Journal
, vol.25
, Issue.8
, pp. 1603-1610
-
-
Ott, M.1
Prestele, M.2
Bauerschmitt, H.3
Funes, S.4
Bonnefoy, N.5
Herrmann, J.M.6
-
165
-
-
0032189225
-
Accumulation of mitochondrially synthesized Saccharomyces cerevisiae Cox2p and Cox3p depends on targeting information in untranslated portions of their mRNAs
-
Sanchirico M. E., Fox T. D., Mason T. L., Accumulation of mitochondrially synthesized Saccharomyces cerevisiae Cox2p and Cox3p depends on targeting information in untranslated portions of their mRNAs EMBO Journal 1998 17 19 5796 5804
-
(1998)
EMBO Journal
, vol.17
, Issue.19
, pp. 5796-5804
-
-
Sanchirico, M.E.1
Fox, T.D.2
Mason, T.L.3
-
166
-
-
0015442410
-
The organization of ribosomal granules within mitochondrial structures of aerobic and anaerobic cells of Saccharomyces cerevisae
-
Watson K., The organization of ribosomal granules within mitochondrial structures of aerobic and anaerobic cells of Saccharomyces cerevisae Journal of Cell Biology 1972 55 3 721 726
-
(1972)
Journal of Cell Biology
, vol.55
, Issue.3
, pp. 721-726
-
-
Watson, K.1
-
167
-
-
0037009132
-
Insertion of proteins into the inner membrane of mitochondria: The role of the Oxa1 complex
-
Stuart R. A., Insertion of proteins into the inner membrane of mitochondria: the role of the Oxa1 complex Biochimica et Biophysica Acta 2002 1592 1 79 87
-
(2002)
Biochimica et Biophysica Acta
, vol.1592
, Issue.1
, pp. 79-87
-
-
Stuart, R.A.1
-
168
-
-
56349118008
-
Roles of Oxa1-related inner-membrane translocases in assembly of respiratory chain complexes
-
Bonnefoy N., Fiumera H. L., Dujardin G., Fox T. D., Roles of Oxa1-related inner-membrane translocases in assembly of respiratory chain complexes Biochimica et Biophysica Acta 2009 1793 1 60 70
-
(2009)
Biochimica et Biophysica Acta
, vol.1793
, Issue.1
, pp. 60-70
-
-
Bonnefoy, N.1
Fiumera, H.L.2
Dujardin, G.3
Fox, T.D.4
-
169
-
-
0348136787
-
Yeast Oxa1 interacts with mitochondrial ribosomes: The importance of the C-terminal region of Oxa1
-
Jia L., Dienhart M., Schramp M., McCauley M., Hell K., Stuart R. A., Yeast Oxa1 interacts with mitochondrial ribosomes: the importance of the C-terminal region of Oxa1 EMBO Journal 2003 22 24 6438 6447
-
(2003)
EMBO Journal
, vol.22
, Issue.24
, pp. 6438-6447
-
-
Jia, L.1
Dienhart, M.2
Schramp, M.3
McCauley, M.4
Hell, K.5
Stuart, R.A.6
-
170
-
-
72449124279
-
Mapping of the saccharomyces cerevisiae oxa1-mitochondrial ribosome interface and identification of MrpL40, a ribosomal protein in close proximity to oxal and critical for oxidative phosphorylation complex assembly
-
Jia L., Kaur J., Stuart R. A., Mapping of the saccharomyces cerevisiae oxa1-mitochondrial ribosome interface and identification of MrpL40, a ribosomal protein in close proximity to oxal and critical for oxidative phosphorylation complex assembly Eukaryotic Cell 2009 8 11 1792 1802
-
(2009)
Eukaryotic Cell
, vol.8
, Issue.11
, pp. 1792-1802
-
-
Jia, L.1
Kaur, J.2
Stuart, R.A.3
-
171
-
-
0035947767
-
Mba1, a novel component of the mitochondrial protein export machinery of the yeast Saccharomyces cerevisiae
-
Preuss M., Leonhard K., Hell K., Stuart R. A., Neupert W., Herrmann J. M., Mba1, a novel component of the mitochondrial protein export machinery of the yeast Saccharomyces cerevisiae Journal of Cell Biology 2001 153 5 1085 1096
-
(2001)
Journal of Cell Biology
, vol.153
, Issue.5
, pp. 1085-1096
-
-
Preuss, M.1
Leonhard, K.2
Hell, K.3
Stuart, R.A.4
Neupert, W.5
Herrmann, J.M.6
-
172
-
-
0347359154
-
LETM1, a gene deleted in Wolf-Hirschhorn syndrome, encodes an evolutionarily conserved mitochondrial protein
-
Schlickum S., Moghekar A., Simpson J. C., LETM1, a gene deleted in Wolf-Hirschhorn syndrome, encodes an evolutionarily conserved mitochondrial protein Genomics 2004 83 2 254 261
-
(2004)
Genomics
, vol.83
, Issue.2
, pp. 254-261
-
-
Schlickum, S.1
Moghekar, A.2
Simpson, J.C.3
-
173
-
-
32644438236
-
Mdm38 interacts with ribosomes and is a component of the mitochondrial protein export machinery
-
Frazier A. E., Taylor R. D., Mick D. U., Mdm38 interacts with ribosomes and is a component of the mitochondrial protein export machinery Journal of Cell Biology 2006 172 4 553 564
-
(2006)
Journal of Cell Biology
, vol.172
, Issue.4
, pp. 553-564
-
-
Frazier, A.E.1
Taylor, R.D.2
Mick, D.U.3
-
174
-
-
65949094376
-
Association of LETM1 and mrpl36 contributes to the regulation of mitochondrial ATP production and necrotic cell death
-
Piao L., Li Y., Kim S. J., Association of LETM1 and mrpl36 contributes to the regulation of mitochondrial ATP production and necrotic cell death Cancer Research 2009 69 8 3397 3404
-
(2009)
Cancer Research
, vol.69
, Issue.8
, pp. 3397-3404
-
-
Piao, L.1
Li, Y.2
Kim, S.J.3
-
175
-
-
50249158546
-
Characterization of the mitochondrial protein LETM1, which maintains the mitochondrial tubular shapes and interacts with the AAA-ATPase BCS1L
-
Tamai S., Iida H., Yokota S., Characterization of the mitochondrial protein LETM1, which maintains the mitochondrial tubular shapes and interacts with the AAA-ATPase BCS1L Journal of Cell Science 2008 121 15 2588 2600
-
(2008)
Journal of Cell Science
, vol.121
, Issue.15
, pp. 2588-2600
-
-
Tamai, S.1
Iida, H.2
Yokota, S.3
-
176
-
-
37849038285
-
LETM1, deleted in Wolf-Hirschhorn syndrome is required for normal mitochondrial morphology and cellular viability
-
Dimmer K. S., Navoni F., Casarin A., LETM1, deleted in Wolf-Hirschhorn syndrome is required for normal mitochondrial morphology and cellular viability Human Molecular Genetics 2008 17 2 201 214
-
(2008)
Human Molecular Genetics
, vol.17
, Issue.2
, pp. 201-214
-
-
Dimmer, K.S.1
Navoni, F.2
Casarin, A.3
-
177
-
-
0027467896
-
Anchoring of DNA to the bacterial cytoplasmic membrane through cotranscriptional synthesis of polypeptides encoding membrane proteins or proteins for export: A mechanism of plasmid hypernegative supercoiling in mutants deficient in DNA topoisomerase i
-
Lynch A. S., Wang J. C., Anchoring of DNA to the bacterial cytoplasmic membrane through cotranscriptional synthesis of polypeptides encoding membrane
-
(1993)
Journal of Bacteriology
, vol.175
, Issue.6
, pp. 1645-1655
-
-
Lynch, A.S.1
Wang, J.C.2
-
178
-
-
0020045584
-
Co-translational insertion of envelope proteins: Theoretical considerations and implications
-
Vos-Scheperkeuter G. H., Witholt B., Co-translational insertion of envelope proteins: theoretical considerations and implications Annales de Microbiologie 1982 133A 1 129 138
-
(1982)
Annales de Microbiologie
, vol.133
, Issue.1
, pp. 129-138
-
-
Vos-Scheperkeuter, G.H.1
Witholt, B.2
-
179
-
-
3142519924
-
A hyperstructure approach to mitochondria
-
Trinei M., Vannier J.-P., Beurton-Aimar M., Norris V., A hyperstructure approach to mitochondria Molecular Microbiology 2004 53 1 41 53
-
(2004)
Molecular Microbiology
, vol.53
, Issue.1
, pp. 41-53
-
-
Trinei, M.1
Vannier, J.-P.2
Beurton-Aimar, M.3
Norris, V.4
-
180
-
-
56349124605
-
Assembly of the oxidative phosphorylation system in humans: What we have learned by studying its defects
-
Fernndez-Vizarra E., Tiranti V., Zeviani M., Assembly of the oxidative phosphorylation system in humans: what we have learned by studying its defects Biochimica et Biophysica Acta 2009 1793 1 200 211
-
(2009)
Biochimica et Biophysica Acta
, vol.1793
, Issue.1
, pp. 200-211
-
-
Fernndez-Vizarra, E.1
Tiranti, V.2
Zeviani, M.3
-
181
-
-
26444488636
-
A molecular chaperone for mitochondrial complex i assembly is mutated in a progressive encephalopathy
-
Ogilvie I., Kennaway N. G., Shoubridge E. A., A molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy Journal of Clinical Investigation 2005 115 10 2784 2792
-
(2005)
Journal of Clinical Investigation
, vol.115
, Issue.10
, pp. 2784-2792
-
-
Ogilvie, I.1
Kennaway, N.G.2
Shoubridge, E.A.3
-
182
-
-
27144528747
-
Human mitochondrial complex i assembly is mediated by NDUFAF1
-
Vogel R. O., Janssen R. J. R. J., Ugalde C., Human mitochondrial complex I assembly is mediated by NDUFAF1 FEBS Journal 2005 272 20 5317 5326
-
(2005)
FEBS Journal
, vol.272
, Issue.20
, pp. 5317-5326
-
-
Vogel, R.O.1
Janssen, R.J.R.J.2
Ugalde, C.3
-
183
-
-
67649639689
-
Baculovirus complementation restores a novel NDUFAF2 mutation causing complex i deficiency
-
Hoefs S. J. G., Dieteren C. E. J., Rodenburg R. J., Baculovirus complementation restores a novel NDUFAF2 mutation causing complex I deficiency Human Mutation 2009 30 7 E728 E736
-
(2009)
Human Mutation
, vol.30
, Issue.7
-
-
Hoefs, S.J.G.1
Dieteren, C.E.J.2
Rodenburg, R.J.3
-
184
-
-
66749128531
-
Mutations in NDUFAF3 (C3ORF60), encoding an NDUFAF4 (C6ORF66)-interacting complex i assembly protein, cause fatal neonatal mitochondrial disease
-
Saada A., Vogel R. O., Hoefs S. J., Mutations in NDUFAF3 (C3ORF60), encoding an NDUFAF4 (C6ORF66)-interacting complex I assembly protein, cause fatal neonatal mitochondrial disease American Journal of Human Genetics 2009 84 6 718 727
-
(2009)
American Journal of Human Genetics
, vol.84
, Issue.6
, pp. 718-727
-
-
Saada, A.1
Vogel, R.O.2
Hoefs, S.J.3
-
186
-
-
33947129099
-
Cytosolic signaling protein Ecsit also localizes to mitochondria where it interacts with chaperone NDUFAF1 and functions in complex i assembly
-
Vogel R. O., Janssen R. J. R. J., van den Brand M. A. M., Cytosolic signaling protein Ecsit also localizes to mitochondria where it interacts with chaperone NDUFAF1 and functions in complex I assembly Genes and Development 2007 21 5 615 624
-
(2007)
Genes and Development
, vol.21
, Issue.5
, pp. 615-624
-
-
Vogel, R.O.1
Janssen, R.J.R.J.2
Van Den Brand, M.A.M.3
-
187
-
-
46349103594
-
A mitochondrial protein compendium elucidates complex i disease biology
-
Pagliarini D. J., Calvo S. E., Chang B., A mitochondrial protein compendium elucidates complex I disease biology Cell 2008 134 1 112 123
-
(2008)
Cell
, vol.134
, Issue.1
, pp. 112-123
-
-
Pagliarini, D.J.1
Calvo, S.E.2
Chang, B.3
-
189
-
-
53049098744
-
Mutation of C20orf7 disrupts complex i assembly and causes lethal neonatal mitochondrial disease
-
Sugiana C., Pagliarini D. J., McKenzie M., Mutation of C20orf7 disrupts complex I assembly and causes lethal neonatal mitochondrial disease American Journal of Human Genetics 2008 83 4 468 478
-
(2008)
American Journal of Human Genetics
, vol.83
, Issue.4
, pp. 468-478
-
-
Sugiana, C.1
Pagliarini, D.J.2
McKenzie, M.3
-
190
-
-
34848911639
-
Human mitochondrial complex i assembly: A dynamic and versatile process
-
Vogel R. O., Smeitink J. A. M., Nijtmans L. G. J., Human mitochondrial complex I assembly: a dynamic and versatile process Biochimica et Biophysica Acta 2007 1767 10 1215 1227
-
(2007)
Biochimica et Biophysica Acta
, vol.1767
, Issue.10
, pp. 1215-1227
-
-
Vogel, R.O.1
Smeitink, J.A.M.2
Nijtmans, L.G.J.3
-
191
-
-
10644244369
-
AIF deficiency compromises oxidative phosphorylation
-
Vahsen N., Cand C., Brire J.-J., AIF deficiency compromises oxidative phosphorylation EMBO Journal 2004 23 23 4679 4689
-
(2004)
EMBO Journal
, vol.23
, Issue.23
, pp. 4679-4689
-
-
Vahsen, N.1
Cand, C.2
Brire, J.-J.3
-
192
-
-
47049126468
-
The iron-sulphur protein Ind1 is required for effective complex i assembly
-
Bych K., Kerscher S., Netz D. J. A., The iron-sulphur protein Ind1 is required for effective complex I assembly EMBO Journal 2008 27 12 1736 1746
-
(2008)
EMBO Journal
, vol.27
, Issue.12
, pp. 1736-1746
-
-
Bych, K.1
Kerscher, S.2
Netz, D.J.A.3
-
193
-
-
67349189168
-
SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy
-
Ghezzi D., Goffrini P., Uziel G., SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy Nature Genetics 2009 41 6 654 656
-
(2009)
Nature Genetics
, vol.41
, Issue.6
, pp. 654-656
-
-
Ghezzi, D.1
Goffrini, P.2
Uziel, G.3
-
194
-
-
17944381521
-
A mutant mitochondrial respiratory chain assembly protein causes complex III deficiency in patients with tubulopathy, encephalopathy and liver failure
-
de Lonlay P., Valnot I., Barrientos A., A mutant mitochondrial respiratory chain assembly protein causes complex III deficiency in patients with tubulopathy, encephalopathy and liver failure Nature Genetics 2001 29 1 57 60
-
(2001)
Nature Genetics
, vol.29
, Issue.1
, pp. 57-60
-
-
De Lonlay, P.1
Valnot, I.2
Barrientos, A.3
-
195
-
-
2342553529
-
Genetic defects of cytochrome c oxidase assembly
-
supplement 1
-
Pecinai P., Houkov H., Hanskov H., Zeman J., Houtk J., Genetic defects of cytochrome c oxidase assembly Physiological Research 2004 53 supplement 1 S213 S223
-
(2004)
Physiological Research
, vol.53
-
-
Pecinai, P.1
Houkov, H.2
Hanskov, H.3
Zeman, J.4
Houtk, J.5
-
196
-
-
0035903210
-
Atp11p and Atp12p are assembly factors for the F1-ATPase in human mitochondria
-
Wang Z.-G., White P. S., Ackerman S. H., Atp11p and Atp12p are assembly factors for the F1-ATPase in human mitochondria Journal of Biological Chemistry 2001 276 33 30773 30778
-
(2001)
Journal of Biological Chemistry
, vol.276
, Issue.33
, pp. 30773-30778
-
-
Wang, Z.-G.1
White, P.S.2
Ackerman, S.H.3
-
197
-
-
33846813499
-
The metalloprotease encoded by ATP23 has a dual function in processing and assembly of subunit 6 of mitochondrial ATPase
-
Zeng X., Neupert W., Tzagoloff A., The metalloprotease encoded by ATP23 has a dual function in processing and assembly of subunit 6 of mitochondrial ATPase Molecular Biology of the Cell 2007 18 2 617 626
-
(2007)
Molecular Biology of the Cell
, vol.18
, Issue.2
, pp. 617-626
-
-
Zeng, X.1
Neupert, W.2
Tzagoloff, A.3
-
198
-
-
35548996344
-
Knockdown of human Oxa1l impairs the biogenesis of F1Fo-ATP synthase and NADH:ubiquinone oxidoreductase
-
Stiburek L., Fornuskova D., Wenchich L., Pejznochova M., Hansikova H., Zeman J., Knockdown of human Oxa1l impairs the biogenesis of F1Fo-ATP synthase and NADH:ubiquinone oxidoreductase Journal of Molecular Biology 2007 374 2 506 516
-
(2007)
Journal of Molecular Biology
, vol.374
, Issue.2
, pp. 506-516
-
-
Stiburek, L.1
Fornuskova, D.2
Wenchich, L.3
Pejznochova, M.4
Hansikova, H.5
Zeman, J.6
-
199
-
-
57049094966
-
The higher level of organization of the oxidative phosphorylation system: Mitochondrial supercomplexes
-
Dudkina N. V., Sunderhaus S., Boekema E. J., Braun H.-P., The higher level of organization of the oxidative phosphorylation system: mitochondrial supercomplexes Journal of Bioenergetics and Biomembranes 2008 40 5 419 424
-
(2008)
Journal of Bioenergetics and Biomembranes
, vol.40
, Issue.5
, pp. 419-424
-
-
Dudkina, N.V.1
Sunderhaus, S.2
Boekema, E.J.3
Braun, H.-P.4
-
200
-
-
57849156466
-
Heavy breathing: Energy conversion by mitochondrial respiratory supercomplexes
-
Schon E. A., Dencher N. A., Heavy breathing: energy conversion by mitochondrial respiratory supercomplexes Cell Metabolism 2009 9 1 1 3
-
(2009)
Cell Metabolism
, vol.9
, Issue.1
, pp. 1-3
-
-
Schon, E.A.1
Dencher, N.A.2
-
201
-
-
55949098781
-
Respiratory active mitochondrial supercomplexes
-
Acn-Prez R., Fernndez-Silva P., Peleato M. L., Prez-Martos A., Enriquez J. A., Respiratory active mitochondrial supercomplexes Molecular Cell 2008 32 4 529 539
-
(2008)
Molecular Cell
, vol.32
, Issue.4
, pp. 529-539
-
-
Acn-Prez, R.1
Fernndez-Silva, P.2
Peleato, M.L.3
Prez-Martos, A.4
Enriquez, J.A.5
-
202
-
-
4344630010
-
Significance of respirasomes for the assembly/stability of human respiratory chain complex i
-
Schgger H., De Coo R., Bauer M. F., Hofmann S., Godino C., Brandt U., Significance of respirasomes for the assembly/stability of human respiratory chain complex I Journal of Biological Chemistry 2004 279 35 36349 36353
-
(2004)
Journal of Biological Chemistry
, vol.279
, Issue.35
, pp. 36349-36353
-
-
Schgger, H.1
De Coo, R.2
Bauer, M.F.3
Hofmann, S.4
Godino, C.5
Brandt, U.6
-
203
-
-
33746327466
-
Mitochondrial respiratory chain supercomplexes are destabilized in Barth Syndrome patients
-
McKenzie M., Lazarou M., Thorburn D. R., Ryan M. T., Mitochondrial respiratory chain supercomplexes are destabilized in Barth Syndrome patients Journal of Molecular Biology 2006 361 3 462 469
-
(2006)
Journal of Molecular Biology
, vol.361
, Issue.3
, pp. 462-469
-
-
McKenzie, M.1
Lazarou, M.2
Thorburn, D.R.3
Ryan, M.T.4
-
204
-
-
27744491193
-
Emerging functions of mammalian mitochondrial fusion and fission
-
Chen H., Chan D. C., Emerging functions of mammalian mitochondrial fusion and fission Human Molecular Genetics 2005 14 2 R283 R289
-
(2005)
Human Molecular Genetics
, vol.14
, Issue.2
-
-
Chen, H.1
Chan, D.C.2
-
205
-
-
67650868959
-
Mitochondrial dynamics in mammalian health and disease
-
Liesa M., Palacin M., Zorzano A., Mitochondrial dynamics in mammalian health and disease Physiological Reviews 2009 89 3 799 845
-
(2009)
Physiological Reviews
, vol.89
, Issue.3
, pp. 799-845
-
-
Liesa, M.1
Palacin, M.2
Zorzano, A.3
-
206
-
-
52449134835
-
Preventing mitochondrial fission impairs mitochondrial function and leads to loss of mitochondrial DNA
-
article e3257
-
Parone P. A., Da Cruz S., Tondera D., Preventing mitochondrial fission impairs mitochondrial function and leads to loss of mitochondrial DNA PLoS One 2008 3 9, article e3257
-
(2008)
PLoS One
, vol.3
, pp. 9
-
-
Parone, P.A.1
Da Cruz, S.2
Tondera, D.3
-
207
-
-
34247525092
-
A lethal defect of mitochondrial and peroxisomal fission
-
Waterham H. R., Koster J., van Roermund C. W. T., Mooyer P. A. W., Wanders R. J. A., Leonard J. V., A lethal defect of mitochondrial and peroxisomal fission New England Journal of Medicine 2007 356 17 1736 1741
-
(2007)
New England Journal of Medicine
, vol.356
, Issue.17
, pp. 1736-1741
-
-
Waterham, H.R.1
Koster, J.2
Van Roermund, C.W.T.3
Mooyer, P.A.W.4
Wanders, R.J.A.5
Leonard, J.V.6
-
208
-
-
0023883150
-
Deletions of muscle mitochondrial DNA in patients with mitochondrial myopathies
-
Holt I. J., Harding A. E., Morgan-Hughes J. A., Deletions of muscle mitochondrial DNA in patients with mitochondrial myopathies Nature 1988 331 6158 717 719
-
(1988)
Nature
, vol.331
, Issue.6158
, pp. 717-719
-
-
Holt, I.J.1
Harding, A.E.2
Morgan-Hughes, J.A.3
-
209
-
-
0024242545
-
Mitochondrial DNA mutation associated with Lebers hereditary optic neuropathy
-
Wallace D. C., Singh G., Lott M. T., Mitochondrial DNA mutation associated with Lebers hereditary optic neuropathy Science 1988 242 4884 1427 1430
-
(1988)
Science
, vol.242
, Issue.4884
, pp. 1427-1430
-
-
Wallace, D.C.1
Singh, G.2
Lott, M.T.3
-
210
-
-
38949120469
-
Human mitochondrial transfer RNAs: Role of pathogenic mutation in disease
-
Scaglia F., Wong L.-J. C., Human mitochondrial transfer RNAs: role of pathogenic mutation in disease Muscle and Nerve 2008 37 2 150 171
-
(2008)
Muscle and Nerve
, vol.37
, Issue.2
, pp. 150-171
-
-
Scaglia, F.1
Wong, L.-J.C.2
-
211
-
-
0013084592
-
Human mitochondrial tRNAs in health and disease
-
Florentz C., Sohm B., Tryoen-Tth P., Ptz J., Sissler M., Human mitochondrial tRNAs in health and disease Cellular and Molecular Life Sciences 2003 60 7 1356 1375
-
(2003)
Cellular and Molecular Life Sciences
, vol.60
, Issue.7
, pp. 1356-1375
-
-
Florentz, C.1
Sohm, B.2
Tryoen-Tth, P.3
Ptz, J.4
Sissler, M.5
-
212
-
-
34447530916
-
Mitochondrial tRNA mutations: Clinical and functional perturbations
-
Zifa E., Giannouli S., Theotokis P., Stamatis C., Mamuris Z., Stathopoulos C., Mitochondrial tRNA mutations: clinical and functional perturbations RNA Biology 2007 4 1 38 66
-
(2007)
RNA Biology
, vol.4
, Issue.1
, pp. 38-66
-
-
Zifa, E.1
Giannouli, S.2
Theotokis, P.3
Stamatis, C.4
Mamuris, Z.5
Stathopoulos, C.6
-
213
-
-
67349191588
-
DNA polymerase gamma and mitochondrial disease: Understanding the consequence of POLG mutations
-
Chan S. S. L., Copeland W. C., DNA polymerase gamma and mitochondrial disease: understanding the consequence of POLG mutations Biochimica et Biophysica Acta 2009 1787 5 312 319
-
(2009)
Biochimica et Biophysica Acta
, vol.1787
, Issue.5
, pp. 312-319
-
-
Chan, S.S.L.1
Copeland, W.C.2
-
214
-
-
33646859687
-
Mutant POLG2 disrupts DNA polymerase subunits and causes progressive external ophthalmoplegia
-
Longley M. J., Clark S., Man C. Y. W., Mutant POLG2 disrupts DNA polymerase subunits and causes progressive external ophthalmoplegia American Journal of Human Genetics 2006 78 6 1026 1034
-
(2006)
American Journal of Human Genetics
, vol.78
, Issue.6
, pp. 1026-1034
-
-
Longley, M.J.1
Clark, S.2
Man, C.Y.W.3
-
215
-
-
33847176234
-
Mitochondrial phosphate-carrier deficiency: A novel disorder of oxidative phosphorylation
-
Mayr J. A., Merkel O., Kohlwein S. D., Mitochondrial phosphate-carrier deficiency: a novel disorder of oxidative phosphorylation American Journal of Human Genetics 2007 80 3 478 484
-
(2007)
American Journal of Human Genetics
, vol.80
, Issue.3
, pp. 478-484
-
-
Mayr, J.A.1
Merkel, O.2
Kohlwein, S.D.3
-
216
-
-
34547736513
-
Deficiency of the subunit of succinate-coenzyme A ligase causes fatal infantile lactic acidosis with mitochondrial DNA depletion
-
Ostergaard E., Christensen E., Kristensen E., Deficiency of the subunit of succinate-coenzyme A ligase causes fatal infantile lactic acidosis with mitochondrial DNA depletion American Journal of Human Genetics 2007 81 2 383 387
-
(2007)
American Journal of Human Genetics
, vol.81
, Issue.2
, pp. 383-387
-
-
Ostergaard, E.1
Christensen, E.2
Kristensen, E.3
-
217
-
-
34249811206
-
Mutation of RRM2B, encoding p53-controlled ribonucleotide reductase (p53R2), causes severe mitochondrial DNA depletion
-
Bourdon A., Minai L., Serre V., Mutation of RRM2B, encoding p53-controlled ribonucleotide reductase (p53R2), causes severe mitochondrial DNA depletion Nature Genetics 2007 39 6 776 780
-
(2007)
Nature Genetics
, vol.39
, Issue.6
, pp. 776-780
-
-
Bourdon, A.1
Minai, L.2
Serre, V.3
-
218
-
-
32644488897
-
Cardiolipin metabolism and Barth Syndrome
-
Hauff K. D., Hatch G. M., Cardiolipin metabolism and Barth Syndrome Progress in Lipid Research 2006 45 2 91 101
-
(2006)
Progress in Lipid Research
, vol.45
, Issue.2
, pp. 91-101
-
-
Hauff, K.D.1
Hatch, G.M.2
-
219
-
-
33744970020
-
Translating m-AAA protease function in mitochondria to hereditary spastic paraplegia
-
Rugarli E. I., Langer T., Translating m-AAA protease function in mitochondria to hereditary spastic paraplegia Trends in Molecular Medicine 2006 12 6 262 269
-
(2006)
Trends in Molecular Medicine
, vol.12
, Issue.6
, pp. 262-269
-
-
Rugarli, E.I.1
Langer, T.2
-
220
-
-
0033569992
-
The diabetes-associated 3243 mutation in the mitochondrial tRNA(Leu(UUR)) gene causes severe mitochondrial dysfunction without a strong decrease in protein synthesis rate
-
Janssen G. M. C., Maassen J. A., van Den Ouweland J. M. W., The diabetes-associated 3243 mutation in the mitochondrial tRNA(Leu(UUR)) gene causes severe mitochondrial dysfunction without a strong decrease in protein synthesis rate Journal of Biological Chemistry 1999 274 42 29744 29748
-
(1999)
Journal of Biological Chemistry
, vol.274
, Issue.42
, pp. 29744-29748
-
-
Janssen, G.M.C.1
Maassen, J.A.2
Van Den Ouweland, J.M.W.3
-
221
-
-
0032697467
-
Molecular phenotype of the np 7472 deafness-associated mitochondrial mutation in osteosarcoma cell cybrids
-
Toompuu M., Tiranti V., Zeviani M., Jacobs H. T., Molecular phenotype of the np 7472 deafness-associated mitochondrial mutation in osteosarcoma cell cybrids Human Molecular Genetics 1999 8 12 2275 2283
-
(1999)
Human Molecular Genetics
, vol.8
, Issue.12
, pp. 2275-2283
-
-
Toompuu, M.1
Tiranti, V.2
Zeviani, M.3
Jacobs, H.T.4
-
222
-
-
0141925696
-
Disorders of mitochondrial protein synthesis
-
Jacobs H. T., Disorders of mitochondrial protein synthesis Human Molecular Genetics 2003 12 2 R293 R301
-
(2003)
Human Molecular Genetics
, vol.12
, Issue.2
-
-
Jacobs, H.T.1
-
223
-
-
34250833548
-
Genetic, pathogenetic, and phenotypic implications of the mitochondrial A3243G tRNALeu(UUR) mutation
-
Finsterer J., Genetic, pathogenetic, and phenotypic implications of the mitochondrial A3243G tRNALeu(UUR) mutation Acta Neurologica Scandinavica 2007 116 1 1 14
-
(2007)
Acta Neurologica Scandinavica
, vol.116
, Issue.1
, pp. 1-14
-
-
Finsterer, J.1
-
224
-
-
0034161959
-
The np 3243 MELAS mutation: Damned if you aminoacylate, damned if you dont
-
Jacobs H. T., Holt I. J., The np 3243 MELAS mutation: damned if you aminoacylate, damned if you dont Human Molecular Genetics 2000 9 4 463 465
-
(2000)
Human Molecular Genetics
, vol.9
, Issue.4
, pp. 463-465
-
-
Jacobs, H.T.1
Holt, I.J.2
-
225
-
-
6344221310
-
Codon-specific translational defect caused by a wobble modification deficiency in mutant tRNA from a human mitochondrial disease
-
Kirino Y., Yasukawa T., Ohta S., Codon-specific translational defect caused by a wobble modification deficiency in mutant tRNA from a human mitochondrial disease Proceedings of the National Academy of Sciences of the United States of America 2004 101 42 15070 15075
-
(2004)
Proceedings of the National Academy of Sciences of the United States of America
, vol.101
, Issue.42
, pp. 15070-15075
-
-
Kirino, Y.1
Yasukawa, T.2
Ohta, S.3
-
226
-
-
0026573082
-
Defects in mitochondrial protein synthesis and respiratory chain activity segregate with the tRNA(Leu(UUR)) mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes
-
King M. P., Koga Y., Davidson M., Schon E. A., Defects in mitochondrial protein synthesis and respiratory chain activity segregate with the tRNA(Leu(UUR)) mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes Molecular and Cellular Biology 1992 12 2 480 490
-
(1992)
Molecular and Cellular Biology
, vol.12
, Issue.2
, pp. 480-490
-
-
King, M.P.1
Koga, Y.2
Davidson, M.3
Schon, E.A.4
-
227
-
-
0029059067
-
MtDNA mutation in MERRF syndrome causes defective aminoacylation of tRNA(Lys) and premature translation termination
-
Enriquez J. A., Chomyn A., Attardi G., MtDNA mutation in MERRF syndrome causes defective aminoacylation of tRNA(Lys) and premature translation termination Nature Genetics 1995 10 1 47 55
-
(1995)
Nature Genetics
, vol.10
, Issue.1
, pp. 47-55
-
-
Enriquez, J.A.1
Chomyn, A.2
Attardi, G.3
-
228
-
-
33947328908
-
Mitochondrial rRNA and tRNA and hearing function
-
Xing G., Chen Z., Cao X., Mitochondrial rRNA and tRNA and hearing function Cell Research 2007 17 3 227 239
-
(2007)
Cell Research
, vol.17
, Issue.3
, pp. 227-239
-
-
Xing, G.1
Chen, Z.2
Cao, X.3
-
229
-
-
0033112848
-
Studies on mitochondrial pathogenesis of Rett syndrome: Ultrastructural data from skin and muscle biopsies and mutational analysis at mtDNA nucleotides 10463 and 2835
-
Cardaioli E., Dotti M. T., Hayek G., Zappella M., Federico A., Studies on mitochondrial pathogenesis of Rett syndrome: ultrastructural data from skin and muscle biopsies and mutational analysis at mtDNA nucleotides 10463 and 2835 Journal of Submicroscopic Cytology and Pathology 1999 31 2 301 304
-
(1999)
Journal of Submicroscopic Cytology and Pathology
, vol.31
, Issue.2
, pp. 301-304
-
-
Cardaioli, E.1
Dotti, M.T.2
Hayek, G.3
Zappella, M.4
Federico, A.5
-
230
-
-
0034926430
-
A novel mutation in the mitochondrial 16S rRNA gene in a patient with MELAS syndrome, diabetes mellitus, hyperthyroidism and cardiomyopathy
-
Hsieh R.-H., Li J.-Y., Pang C.-Y., Wei Y.-H., A novel mutation in the mitochondrial 16S rRNA gene in a patient with MELAS syndrome, diabetes mellitus, hyperthyroidism and cardiomyopathy Journal of Biomedical Science 2001 8 4 328 335
-
(2001)
Journal of Biomedical Science
, vol.8
, Issue.4
, pp. 328-335
-
-
Hsieh, R.-H.1
Li, J.-Y.2
Pang, C.-Y.3
Wei, Y.-H.4
-
231
-
-
0027200741
-
Mitochondrial DNA variants observed in Alzheimer disease and Parkinson disease patients
-
Shoffner J. M., Brown M. D., Torroni A., Mitochondrial DNA variants observed in Alzheimer disease and Parkinson disease patients Genomics 1993 17 1 171 184
-
(1993)
Genomics
, vol.17
, Issue.1
, pp. 171-184
-
-
Shoffner, J.M.1
Brown, M.D.2
Torroni, A.3
-
233
-
-
34249008188
-
Pleiotropic effects and compensation mechanisms determine tissue specificity in mitochondrial myopathy and sideroblastic anemia (MLASA)
-
Bykhovskaya Y., Mengesha E., Fischel-Ghodsian N., Pleiotropic effects and compensation mechanisms determine tissue specificity in mitochondrial myopathy and sideroblastic anemia (MLASA) Molecular Genetics and Metabolism 2007 91 2 148 156
-
(2007)
Molecular Genetics and Metabolism
, vol.91
, Issue.2
, pp. 148-156
-
-
Bykhovskaya, Y.1
Mengesha, E.2
Fischel-Ghodsian, N.3
-
234
-
-
0344026255
-
Single atom modification (O → S) of tRNA confers ribosome binding
-
Ashraf S. S., Sochacka E., Cain R., Guenther R., Malkiewicz A., Agris P. F., Single atom modification (O → S) of tRNA confers ribosome binding RNA 1999 5 2 188 194
-
(1999)
RNA
, vol.5
, Issue.2
, pp. 188-194
-
-
Ashraf, S.S.1
Sochacka, E.2
Cain, R.3
Guenther, R.4
Malkiewicz, A.5
Agris, P.F.6
-
235
-
-
0034619490
-
Modified nucleoside dependent Watson-Crick and wobble codon binding by tRNA(Lys)(UUU) species
-
Yarian C., Marszalek M., Sochacka E., Modified nucleoside dependent Watson-Crick and wobble codon binding by tRNA(Lys)(UUU) species Biochemistry 2000 39 44 13390 13395
-
(2000)
Biochemistry
, vol.39
, Issue.44
, pp. 13390-13395
-
-
Yarian, C.1
Marszalek, M.2
Sochacka, E.3
-
236
-
-
67651097815
-
The R336Q mutation in human mitochondrial EFTu prevents the formation of an active mt-EFTu GTP aa-tRNA ternary complex
-
Valente L., Shigi N., Suzuki T., Zeviani M., The R336Q mutation in human mitochondrial EFTu prevents the formation of an active mt-EFTu GTP aa-tRNA ternary complex Biochimica et Biophysica Acta 2009 1792 8 791 795
-
(2009)
Biochimica et Biophysica Acta
, vol.1792
, Issue.8
, pp. 791-795
-
-
Valente, L.1
Shigi, N.2
Suzuki, T.3
Zeviani, M.4
-
237
-
-
33846021292
-
Tissue-specific differences in human transfer RNA expression
-
article e221
-
Dittmar K. A., Goodenbour J. M., Pan T., Tissue-specific differences in human transfer RNA expression PLoS Genetics 2006 2 12, article e221
-
(2006)
PLoS Genetics
, vol.2
, pp. 12
-
-
Dittmar, K.A.1
Goodenbour, J.M.2
Pan, T.3
-
238
-
-
0029036107
-
Mitochondrial and cytoplasmic isoleucyl-, glutamyl-and arginyl-tRNA synthetases of yeast are encoded by separate genes
-
Tzagoloff A., Shtanko A., Mitochondrial and cytoplasmic isoleucyl-, glutamyl-and arginyl-tRNA synthetases of yeast are encoded by separate genes European Journal of Biochemistry 1995 230 2 582 586
-
(1995)
European Journal of Biochemistry
, vol.230
, Issue.2
, pp. 582-586
-
-
Tzagoloff, A.1
Shtanko, A.2
-
239
-
-
42049097275
-
A clinical, genetic, and biochemical characterization of SPG7 mutations in a large cohort of patients with hereditary spastic paraplegia
-
Arnoldi A., Tonelli A., Crippa F., A clinical, genetic, and biochemical characterization of SPG7 mutations in a large cohort of patients with hereditary spastic paraplegia Human Mutation 2008 29 4 522 531
-
(2008)
Human Mutation
, vol.29
, Issue.4
, pp. 522-531
-
-
Arnoldi, A.1
Tonelli, A.2
Crippa, F.3
-
240
-
-
0344736798
-
Loss of m-AAA protease in mitochondria causes complex i deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia
-
Atorino L., Silvestri L., Koppen M., Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia Journal of Cell Biology 2003 163 4 777 787
-
(2003)
Journal of Cell Biology
, vol.163
, Issue.4
, pp. 777-787
-
-
Atorino, L.1
Silvestri, L.2
Koppen, M.3
-
241
-
-
2442542546
-
A clinical, genetic and biochemical study of SPG7 mutations in hereditary spastic paraplegia
-
Wilkinson P. A., Crosby A. H., Turner C., A clinical, genetic and biochemical study of SPG7 mutations in hereditary spastic paraplegia Brain 2004 127 5 973 980
-
(2004)
Brain
, vol.127
, Issue.5
, pp. 973-980
-
-
Wilkinson, P.A.1
Crosby, A.H.2
Turner, C.3
-
242
-
-
33846127778
-
Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia
-
Koppen M., Metodiev M. D., Casari G., Rugarli E. I., Langer T., Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia Molecular and Cellular Biology 2007 27 2 758 767
-
(2007)
Molecular and Cellular Biology
, vol.27
, Issue.2
, pp. 758-767
-
-
Koppen, M.1
Metodiev, M.D.2
Casari, G.3
Rugarli, E.I.4
Langer, T.5
-
243
-
-
10744232283
-
Ethylmalonic encephalopathy is caused by mutations in ETHE1, a gene encoding a mitochondrial matrix protein
-
Tiranti V., DAdamo P., Briem E., Ethylmalonic encephalopathy is caused by mutations in ETHE1, a gene encoding a mitochondrial matrix protein American Journal of Human Genetics 2004 74 2 239 252
-
(2004)
American Journal of Human Genetics
, vol.74
, Issue.2
, pp. 239-252
-
-
Tiranti, V.1
Dadamo, P.2
Briem, E.3
-
244
-
-
67349209853
-
Next-generation DNA sequencing techniques
-
Ansorge W. J., Next-generation DNA sequencing techniques New Biotechnology 2009 25 4 195 203
-
(2009)
New Biotechnology
, vol.25
, Issue.4
, pp. 195-203
-
-
Ansorge, W.J.1
-
245
-
-
68249092574
-
Massively parallel sequencing: The next big thing in genetic medicine
-
Tucker T., Marra M., Friedman J. M., Massively parallel sequencing: the next big thing in genetic medicine American Journal of Human Genetics 2009 85 2 142 154
-
(2009)
American Journal of Human Genetics
, vol.85
, Issue.2
, pp. 142-154
-
-
Tucker, T.1
Marra, M.2
Friedman, J.M.3
-
246
-
-
70249111091
-
Targeted capture and massively parallel sequencing of 12 human exomes
-
Ng S. B., Turner E. H., Robertson P. D., Targeted capture and massively parallel sequencing of 12 human exomes Nature 2009 461 7261 272 276
-
(2009)
Nature
, vol.461
, Issue.7261
, pp. 272-276
-
-
Ng, S.B.1
Turner, E.H.2
Robertson, P.D.3
-
247
-
-
35748953750
-
Multiplex amplification of large sets of human exons
-
Porreca G. J., Zhang K., Li J. B., Multiplex amplification of large sets of human exons Nature Methods 2007 4 11 931 936
-
(2007)
Nature Methods
, vol.4
, Issue.11
, pp. 931-936
-
-
Porreca, G.J.1
Zhang, K.2
Li, J.B.3
-
248
-
-
33746525415
-
Systematic mapping of genetic interactions in Caenorhabditis elegans identifies common modifiers of diverse signaling pathways
-
Lehner B., Crombie C., Tischler J., Fortunato A., Fraser A. G., Systematic mapping of genetic interactions in Caenorhabditis elegans identifies common modifiers of diverse signaling pathways Nature Genetics 2006 38 8 896 903
-
(2006)
Nature Genetics
, vol.38
, Issue.8
, pp. 896-903
-
-
Lehner, B.1
Crombie, C.2
Tischler, J.3
Fortunato, A.4
Fraser, A.G.5
-
249
-
-
34250676955
-
Mitochondrial diseases: Therapeutic approaches
-
DiMauro S., Mancuso M., Mitochondrial diseases: therapeutic approaches Bioscience Reports 2007 27 13 125 137
-
(2007)
Bioscience Reports
, vol.27
, Issue.13
, pp. 125-137
-
-
Dimauro, S.1
Mancuso, M.2
-
250
-
-
58149349815
-
Mitochondrial medicine: Entering the era of treatment
-
Koene S., Smeitink J., Mitochondrial medicine: entering the era of treatment Journal of Internal Medicine 2009 265 2 193 209
-
(2009)
Journal of Internal Medicine
, vol.265
, Issue.2
, pp. 193-209
-
-
Koene, S.1
Smeitink, J.2
|