-
1
-
-
0025666322
-
Leu(UUR) gene associated with the MELAS subgroup of mitochondrial encephalomyopathies
-
Goto, Y., Nonaka, I. and Horai, S. (1990) A mutation in the tRNA(Leu)(UUR) gene associated with the MELAS subgroup of mitochondrial encephalomyopathies. Nature, 348, 651-653. (Pubitemid 120015131)
-
(1990)
Nature
, vol.348
, Issue.6302
, pp. 651-653
-
-
Goto, Y.-I.1
Nonaka, I.2
Horai, S.3
-
2
-
-
0033862962
-
The epidemiology of pathogenic mitochondrial DNA mutations
-
DOI 10.1002/1531-8249(200008)48:2<188::AID-ANA8>3.0.CO;2-P
-
Chinnery, P.F., Johnson, M.A., Wardell, T.M., Singh-Kler, R., Hayes, C., Brown, D.T., Taylor, R.W., Bindoff, L.A. and Turnbull, D.M. (2000) The epidemiology of pathogenic mitochondrial DNA mutations. Ann. Neurol., 48, 188-193. (Pubitemid 30617037)
-
(2000)
Annals of Neurology
, vol.48
, Issue.2
, pp. 188-193
-
-
Chinnery, P.F.1
Johnson, M.A.2
Wardell, T.M.3
Singh-Kler, R.4
Hayes, C.5
Brown, D.T.6
Taylor, R.W.7
Bindoff, L.A.8
Turnbull, D.M.9
-
3
-
-
0032231623
-
Epidemiology of A3243G, the mutation for mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes: Prevalence of the mutation in an adult population
-
DOI 10.1086/301959
-
Majamaa, K., Moilanen, J.S., Uimonen, S., Remes, A.M., Salmela, P.I., Karppa, M., Majamaa-Voltti, K.A., Rusanen, H., Sorri, M., Peuhkurinen, K.J. et al. (1998) Epidemiology of A3243G, the mutation for mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes: prevalence of the mutation in an adult population. Ma. J. Hum. Genet., 63, 447-454. (Pubitemid 30418626)
-
(1998)
American Journal of Human Genetics
, vol.63
, Issue.2
, pp. 447-454
-
-
Majamaa, K.1
Moilanen, J.S.2
Uimonen, S.3
Remes, A.M.4
Salmela, P.I.5
Karppa, M.6
Majamaa-Voltti, K.A.M.7
Rusanen, H.8
Sorri, M.9
Peuhkurinen, K.J.10
Hassinen, I.E.11
-
4
-
-
34948867248
-
Prevalence, segregation, and phenotype of the mitochondrial DNA 3243A>G mutation in children
-
DOI 10.1002/ana.21196
-
Uusimaa, J., Moilanen, J.S., Vainionpaa, L., Tapanainen, P., Lindholm, P., Nuutinen, M., Lopponen, T., Maki-Torkko, E., Rantala, H. and Majamaa, K. (2007) Prevalence, segregation, and phenotype of the mitochondrial DNA 3243AG mutation in children. Ann. Neurol., 62, 278-287. (Pubitemid 47525404)
-
(2007)
Annals of Neurology
, vol.62
, Issue.3
, pp. 278-287
-
-
Uusimaa, J.1
Moilanen, J.S.2
Vainionpaa, L.3
Tapanainen, P.4
Lindholm, P.5
Nuutinen, M.6
Lopponen, T.7
Maki-Torkko, E.8
Rantala, H.9
Majamaa, K.10
-
5
-
-
33846094306
-
An enhanced MITOMAP with a global mtDNA mutational phylogeny
-
DOI 10.1093/nar/gkl927
-
Ruiz-Pesini, E., Lott, M.T., Procaccio, V., Poole, J.C., Brandon, M.C., Mishmar, D., Yi, C., Kreuziger, J., Baldi, P. and Wallace, D.C. (2007) An enhanced MITOMAP with a global mtDNA mutational phylogeny. Nucleic Acids Res., 35, D823-D828. (Pubitemid 46056316)
-
(2007)
Nucleic Acids Research
, vol.35
, Issue.SUPPL. 1
-
-
Ruiz-Pesini, E.1
Lott, M.T.2
Procaccio, V.3
Poole, J.C.4
Brandon, M.C.5
Mishmar, D.6
Yi, C.7
Kreuziger, J.8
Baldi, P.9
Wallace, D.C.10
-
6
-
-
4644269393
-
Varying loads of the mitochondrial DNA A3243G mutation in different tissues: Implications for diagnosis
-
DOI 10.1002/ajmg.a.30220
-
Shanske, S., Pancrudo, J., Kaufmann, P., Engelstad, K., Jhung, S., Lu, J., Naini, A., DiMauro, S. and De Vivo, D.C. (2004) Varying loads of the mitochondrial DNA A3243G mutation in different tissues: implications for diagnosis. Am. J. Med. Genet., 130A, 134-137. (Pubitemid 39303546)
-
(2004)
American Journal of Medical Genetics
, vol.130
, Issue.2
, pp. 134-137
-
-
Shanske, S.1
Pancrudo, J.2
Kaufmann, P.3
Engelstad, K.4
Jhung, S.5
Lu, J.6
Naini, A.7
DiMauro, S.8
De Vivo, D.C.9
-
7
-
-
42749083478
-
The impact of mitochondrial tRNA mutations on the amount of ATP synthase differs in the brain compared to other tissues
-
Fornuskova, D., Brantova, O., Tesarova, M., Stiburek, L., Honzik, T., Wenchich, L., Tietzeova, E., Hansikova, H. and Zeman, J. (2008) The impact of mitochondrial tRNA mutations on the amount of ATP synthase differs in the brain compared to other tissues. Biochim. Biophys. Acta, 1782, 317-325.
-
(2008)
Biochim. Biophys. Acta
, vol.1782
, pp. 317-325
-
-
Fornuskova, D.1
Brantova, O.2
Tesarova, M.3
Stiburek, L.4
Honzik, T.5
Wenchich, L.6
Tietzeova, E.7
Hansikova, H.8
Zeman, J.9
-
8
-
-
0033950567
-
Oxidative phosphorylation defect in the brains of carriers of the tRNA(leu(UUR)) A3243G mutation in a MELAS pedigree
-
DOI 10.1002/1531-8249(200002)47:2<179::AID-ANA7>3.0.CO;2-Z
-
Dubeau, F., De Stefano, N., Zifkin, B.G., Arnold, D.L. and Shoubridge, E.A. (2000) Oxidative phosphorylation defect in the brains of carriers of the tRNAleu(UUR) A3243G mutation in a MELAS pedigree. Ann. Neurol., 47, 179-185. (Pubitemid 30078564)
-
(2000)
Annals of Neurology
, vol.47
, Issue.2
, pp. 179-185
-
-
Dubeau, F.1
De Stefano, N.2
Zifkin, B.G.3
Arnold, D.L.4
Shoubridge, E.A.5
-
9
-
-
0342470992
-
Decreased aminoacylation of mutant tRNAs in MELAS but not in MERRF patients
-
Borner, G.V., Zeviani, M., Tiranti, V., Carrara, F., Hoffmann, S., Gerbitz, K.D., Lochmuller, H., Pongratz, D., Klopstock, T., Melberg, A. et al. (2000) Decreased aminoacylation of mutant tRNAs in MELAS but not in MERRF patients. Hum. Mol. Genet., 9, 467-475. (Pubitemid 30154005)
-
(2000)
Human Molecular Genetics
, vol.9
, Issue.4
, pp. 467-475
-
-
Borner, G.V.1
Zeviani, M.2
Tiranti, V.3
Carrara, F.4
Hoffmann, S.5
Gerbitz, K.D.6
Lochmuller, H.7
Pongratz, D.8
Klopstock, T.9
Melberg, A.10
Holme, E.11
Paabo, S.12
-
10
-
-
0034705419
-
The mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke- like episode syndrome-associated human mitochondrial tRNA(Leu(UUR)) mutation causes aminoacylation deficiency and concomitant reduced association of mRNA with ribosomes
-
DOI 10.1074/jbc.M908734199
-
Chomyn, A., Enriquez, J.A., Micol, V., Fernandez-Silva, P. and Attardi, G. (2000) The mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episode syndrome-associated human mitochondrial tRNALeu(UUR) mutation causes aminoacylation deficiency and concomitant reduced association of mRNA with ribosomes. J. Biol. Chem., 275, 19198-19209. (Pubitemid 30422891)
-
(2000)
Journal of Biological Chemistry
, vol.275
, Issue.25
, pp. 19198-19209
-
-
Chomyn, A.1
Enriquez, J.A.2
Micol, V.3
Fernandez-Silva, P.4
Attardi, G.5
-
11
-
-
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 stroke like episodes
-
King, M.P., Koga, Y., Davidson, M. and Schon, E.A. (1992) Defects in mitochondrial protein synthesis and respiratory chain activity segregate with the tRNA(Leu(UUR)) mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke like episodes. Mol. Cell Biol., 12, 480-490.
-
(1992)
Mol. Cell Biol.
, vol.12
, pp. 480-490
-
-
King, M.P.1
Koga, Y.2
Davidson, M.3
Schon, E.A.4
-
12
-
-
0031895748
-
A tRNA suppressor mutation in human mitochondria
-
DOI 10.1038/ng0498-350
-
El Meziane, A., Lehtinen, S.K., Hance, N., Nijtmans, L.G., Dunbar, D., Holt, I.J. and Jacobs, H.T. (1998) A tRNA suppressor mutation in human mitochondria. Nature Genet., 18, 350-353. (Pubitemid 28158163)
-
(1998)
Nature Genetics
, vol.18
, Issue.4
, pp. 350-353
-
-
El Meziane, A.1
Lehtinen, S.K.2
Hance, N.3
Nijtmans, L.G.J.4
Dunbar, D.5
Holt, I.J.6
Jacobs, H.T.7
-
13
-
-
0037417764
-
Leu(UUR) decreases the efficiency of aminoacylation
-
DOI 10.1021/bi026882r
-
Park, H., Davidson, E. and King, M.P. (2003) The pathogenic A3243G mutation in human mitochondrial tRNALeu(UUR) decreases the efficiency of aminoacylation. Biochemistry, 42, 958-964. (Pubitemid 36159528)
-
(2003)
Biochemistry
, vol.42
, Issue.4
, pp. 958-964
-
-
Park, H.1
Davidson, E.2
King, M.P.3
-
14
-
-
0033081419
-
Search for differences in post-transcriptional modification patterns of mitochondrial DNA-encoded wild-type and mutant human tRNA(Lys) and tRNA(Leu(UUR))
-
DOI 10.1093/nar/27.3.756
-
Helm, M., Florentz, C., Chomyn, A. and Attardi, G. (1999) Search for differences in post-transcriptional modification patterns of mitochondrial DNA-encoded wild-type and mutant human tRNALys and tRNALeu(UUR). Nucleic Acids Res., 27, 756-763. (Pubitemid 29209362)
-
(1999)
Nucleic Acids Research
, vol.27
, Issue.3
, pp. 756-763
-
-
Helm, M.1
Florentz, C.2
Chomyn, A.3
Attardi, G.4
-
15
-
-
6344221310
-
Codon-specific translational defect caused by a wobble modification deficiency in mutant tRNA from a human mitochondrial disease
-
DOI 10.1073/pnas.0405173101
-
Kirino, Y., Yasukawa, T., Ohta, S., Akira, S., Ishihara, K., Watanabe, K. and Suzuki, T. (2004) Codon-specific translational defect caused by a wobble modification deficiency in mutant tRNA from a human mitochondrial disease. Proc. Natl Acad. Sci. USA, 101, 15070-15075. (Pubitemid 39391720)
-
(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
Akira, S.4
Ishihara, K.5
Watanabe, K.6
Suzuki, T.7
-
16
-
-
21144455795
-
Wobble modification deficiency in mutant tRNAs in patients with mitochondrial diseases
-
DOI 10.1016/j.febslet.2005.04.038, PII S0014579305005132
-
Yasukawa, T., Kirino, Y., Ishii, N., Holt, I.J., Jacobs, H.T., Makifuchi, T., Fukuhara, N., Ohta, S., Suzuki, T. and Watanabe, K. (2005) Wobble modification deficiency in mutant tRNAs in patients with mitochondrial diseases. FEBS Lett., 579, 2948-2952. (Pubitemid 40725272)
-
(2005)
FEBS Letters
, vol.579
, Issue.13
, pp. 2948-2952
-
-
Yasukawa, T.1
Kirino, Y.2
Ishii, N.3
Holt, I.J.4
Jacobs, H.T.5
Makifuchi, T.6
Fukuhara, N.7
Ohta, S.8
Suzuki, T.9
Watanabe, K.10
-
17
-
-
0034635519
-
Modification defect at anticodon wobble nucleotide of mitochondrial tRNAs(Leu)(UUR) with pathogenic mutations of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes
-
DOI 10.1074/jbc.275.6.4251
-
Yasukawa, T., Suzuki, T., Ueda, T., Ohta, S. and Watanabe, K. (2000) Modification defect at anticodon wobble nucleotide of mitochondrial tRNAs(Leu)(UUR) with pathogenic mutations of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes. J. Biol. Chem., 275, 4251-4257. (Pubitemid 30094664)
-
(2000)
Journal of Biological Chemistry
, vol.275
, Issue.6
, pp. 4251-4257
-
-
Yasukawa, T.1
Suzuki, T.2
Suzuki, T.3
Ueda, T.4
Ohta, S.5
Watanabe, K.6
-
18
-
-
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. and Shoubridge, E.A. (2008) 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. Hum. Mol. Genet., 17, 3697-3707.
-
(2008)
Hum. Mol. Genet.
, vol.17
, pp. 3697-3707
-
-
Sasarman, F.1
Antonicka, H.2
Shoubridge, E.A.3
-
19
-
-
0029790507
-
Altered mitochondrial function in fibroblasts containing MELAS or MERRF mitochondrial DNA mutations
-
James, A.M., Wei, Y.H., Pang, C.Y. and Murphy, M.P. (1996) Altered mitochondrial function in fibroblasts containing MELAS or MERRF mitochondrial DNA mutations. Biochem. J., 318 (Pt 2), 401-407. (Pubitemid 26305937)
-
(1996)
Biochemical Journal
, vol.318
, Issue.2
, pp. 401-407
-
-
James, A.M.1
Wei, Y.-H.2
Pang, C.-Y.3
Murphy, M.P.4
-
20
-
-
0030059913
-
Complex I deficiency is associated with 3243G:C mitochondrial DNA in osteosarcoma cell cybrids
-
DOI 10.1093/hmg/5.1.123
-
Dunbar, D.R., Moonie, P.A., Zeviani, M. and Holt, I.J. (1996) Complex I deficiency is associated with 3243G:C mitochondrial DNA in osteosarcoma cell cybrids. Hum. Mol. Genet., 5, 123-129. (Pubitemid 26018283)
-
(1996)
Human Molecular Genetics
, vol.5
, Issue.1
, pp. 123-129
-
-
Dunbar, D.R.1
Moonie, P.A.2
Zeviani, M.3
Holt, I.J.4
-
21
-
-
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., Daga, A., Hurko, O., Johns, D., Lai, S.T., Nonaka, I., Angelini, C. and Attardi, G. (1992) 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. Proc. Natl Acad. Sci. USA, 89, 4221-4225.
-
(1992)
Proc. Natl Acad. Sci. USA
, vol.89
, pp. 4221-4225
-
-
Chomyn, A.1
Martinuzzi, A.2
Yoneda, M.3
Daga, A.4
Hurko, O.5
Johns, D.6
Lai, S.T.7
Nonaka, I.8
Angelini, C.9
Attardi, G.10
-
22
-
-
0030779110
-
Pathophysiology of the MELAS 3243 transition mutation
-
DOI 10.1074/jbc.272.43.27189
-
Flierl, A., Reichmann, H. and Seibel, P. (1997) Pathophysiology of the MELAS 3243 transition mutation. J. Biol. Chem., 272, 27189-27196. (Pubitemid 27452678)
-
(1997)
Journal of Biological Chemistry
, vol.272
, Issue.43
, pp. 27189-27196
-
-
Flierl, A.1
Reichmann, H.2
Seibel, P.3
-
23
-
-
34848919548
-
Leu(UUR) mutation induces mitochondrial dysfunction and variable disease expression without dominant negative acting translational defects in complex IV subunits at UUR codons
-
DOI 10.1093/hmg/ddm203
-
Janssen, G.M., Hensbergen, P.J., van Bussel, F.J., Balog, C.I., Maassen, J.A., Deelder, A.M. and Raap, A.K. (2007) The A3243G tRNALeu(UUR) mutation induces mitochondrial dysfunction and variable disease expression without dominant negative acting translational defects in complex IV subunits at UUR codons. Hum. Mol. Genet., 16, 2472-2481. (Pubitemid 47500633)
-
(2007)
Human Molecular Genetics
, vol.16
, Issue.20
, pp. 2472-2481
-
-
Janssen, G.M.C.1
Hensbergen, P.J.2
Van Bussel, F.J.3
Balog, C.I.A.4
Maassen, J.A.5
Deelder, A.M.6
Raap, A.K.7
-
24
-
-
0042422222
-
Nutritional cofactor treatment in mitochondrial disorders
-
DOI 10.1016/S0002-8223(03)00476-0, PII S0002822303004760
-
Marriage, B., Clandinin, M.T. and Glerum, D.M. (2003) Nutritional cofactor treatment in mitochondrial disorders. J. Am. Dietetic Assoc., 103, 1029-1038. (Pubitemid 44348972)
-
(2003)
Journal of the American Dietetic Association
, vol.103
, Issue.8
, pp. 1029-1038
-
-
Marriage, B.1
Clandinin, M.T.2
Glerum, D.M.3
-
25
-
-
70349284435
-
Mitochondrial gene replacement in primate offspring and embryonic stem cells
-
Tachibana, M., Sparman, M., Sritanaudomchai, H., Ma, H., Clepper, L., Woodward, J., Li, Y., Ramsey, C., Kolotushkina, O. and Mitalipov, S. (2009) Mitochondrial gene replacement in primate offspring and embryonic stem cells. Nature, 461, 367-372.
-
(2009)
Nature
, vol.461
, pp. 367-372
-
-
Tachibana, M.1
Sparman, M.2
Sritanaudomchai, H.3
Ma, H.4
Clepper, L.5
Woodward, J.6
Li, Y.7
Ramsey, C.8
Kolotushkina, O.9
Mitalipov, S.10
-
26
-
-
77952096877
-
Pronuclear transfer in human embryos to prevent transmission of mitochondrial DNA disease
-
Craven, L., Tuppen, H.A., Greggains, G.D., Harbottle, S.J., Murphy, J.L., Cree, L.M., Murdoch, A.P., Chinnery, P.F., Taylor, R.W., Lightowlers, R.N. et al. (2010) Pronuclear transfer in human embryos to prevent transmission of mitochondrial DNA disease. Nature, 465, 82-85.
-
(2010)
Nature
, vol.465
, pp. 82-85
-
-
Craven, L.1
Tuppen, H.A.2
Greggains, G.D.3
Harbottle, S.J.4
Murphy, J.L.5
Cree, L.M.6
Murdoch, A.P.7
Chinnery, P.F.8
Taylor, R.W.9
Lightowlers, R.N.10
-
27
-
-
55549087221
-
Overexpressed mitochondrial leucyl-tRNA synthetase suppresses the A3243G mutation in the mitochondrial tRNA(Leu(UUR)) gene
-
Park, H., Davidson, E. and King, M.P. (2008) Overexpressed mitochondrial leucyl-tRNA synthetase suppresses the A3243G mutation in the mitochondrial tRNA(Leu(UUR)) gene. RNA, 14, 2407-2416.
-
(2008)
RNA
, vol.14
, pp. 2407-2416
-
-
Park, H.1
Davidson, E.2
King, M.P.3
-
28
-
-
77950653171
-
Human mitochondrial leucyl-tRNA synthetase corrects mitochondrial dysfunctions due to the tRNALeu(UUR) A3243G mutation, associated with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like symptoms and diabetes
-
Li, R. and Guan, M.X. (2010) Human mitochondrial leucyl-tRNA synthetase corrects mitochondrial dysfunctions due to the tRNALeu(UUR) A3243G mutation, associated with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like symptoms and diabetes. Mol. Cell Biol., 30, 2147-2154.
-
(2010)
Mol. Cell Biol.
, vol.30
, pp. 2147-2154
-
-
Li, R.1
Guan, M.X.2
-
29
-
-
0346666699
-
The yeast counterparts of human 'MELAS' mutations cause mitochondrial dysfunction that can be rescued by overexpression of the mitochondrial translation factor EF-Tu
-
DOI 10.1038/sj.embor.embor713
-
Feuermann, M., Francisci, S., Rinaldi, T., De Luca, C., Rohou, H., Frontali, L. and Bolotin-Fukuhara, M. (2003) The yeast counterparts of human 'MELAS' mutations cause mitochondrial dysfunction that can be rescued by overexpression of the mitochondrial translation factor EF-Tu. EMBO Rep., 4, 53-58. (Pubitemid 36305378)
-
(2003)
EMBO Reports
, vol.4
, Issue.1
, pp. 53-58
-
-
Feuermann, M.1
Francisci, S.2
Rinaldi, T.3
De Luca, C.4
Rohou, H.5
Frontali, L.6
Bolotin-Fukuhara, M.7
-
30
-
-
0031690491
-
Evidence for the presence of 5S rRNA in mammalian mitochondria
-
Magalhaes, P.J., Andreu, A.L. and Schon, E.A. (1998) Evidence for the presence of 5S rRNA in mammalian mitochondria. Mol. Biol. Cell, 9, 2375-2382. (Pubitemid 28425882)
-
(1998)
Molecular Biology of the Cell
, vol.9
, Issue.9
, pp. 2375-2382
-
-
Magalhaes, P.J.1
Andreu, A.L.2
Schon, E.A.3
-
31
-
-
0035824634
-
5 S rRNA and tRNA import into human mitochondria. Comparison of in vitro requirements
-
Entelis, N.S., Kolesnikova, O.A., Dogan, S., Martin, R.P. and Tarassov, I.A. (2001) 5 S rRNA and tRNA import into human mitochondria. Comparison of in vitro requirements. J. Biol. Chem., 276, 45642-45653.
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 45642-45653
-
-
Entelis, N.S.1
Kolesnikova, O.A.2
Dogan, S.3
Martin, R.P.4
Tarassov, I.A.5
-
32
-
-
41649095551
-
Two distinct structural elements of 5S rRNA are needed for its import into human mitochondria
-
DOI 10.1261/rna.952208
-
Smirnov, A., Tarassov, I., Mager-Heckel, A.M., Letzelter, M., Martin, R.P., Krasheninnikov, I.A. and Entelis, N. (2008) Two distinct structural elements of 5S rRNA are needed for its import into human mitochondria. RNA, 14, 749-759. (Pubitemid 351480886)
-
(2008)
RNA
, vol.14
, Issue.4
, pp. 749-759
-
-
Smirnov, A.1
Tarassov, I.2
Mager-Heckel, A.-M.3
Letzelter, M.4
Martin, R.P.5
Krasheninnikov, I.A.6
Entelis, N.7
-
33
-
-
48249128388
-
Mammalian mitochondria have the innate ability to import tRNAs by a mechanism distinct from protein import
-
Rubio, M.A., Rinehart, J.J., Krett, B., Duvezin-Caubet, S., Reichert, A.S., Soll, D. and Alfonzo, J.D. (2008) Mammalian mitochondria have the innate ability to import tRNAs by a mechanism distinct from protein import. Proc. Natl Acad. Sci. USA, 105, 9186-9191.
-
(2008)
Proc. Natl Acad. Sci. USA
, vol.105
, pp. 9186-9191
-
-
Rubio, M.A.1
Rinehart, J.J.2
Krett, B.3
Duvezin-Caubet, S.4
Reichert, A.S.5
Soll, D.6
Alfonzo, J.D.7
-
34
-
-
77957252102
-
Mitochondrial enzyme rhodanese is essential for 5 S ribosomal RNA import into human mitochondria
-
Smirnov, A., Comte, C., Mager-Heckel, A.M., Addis, V., Krasheninnikov, I.A., Martin, R.P., Entelis, N. and Tarassov, I. (2010) Mitochondrial enzyme rhodanese is essential for 5 S ribosomal RNA import into human mitochondria. J. Biol. Chem., 285, 30792-30803.
-
(2010)
J. Biol. Chem.
, vol.285
, pp. 30792-30803
-
-
Smirnov, A.1
Comte, C.2
Mager-Heckel, A.M.3
Addis, V.4
Krasheninnikov, I.A.5
Martin, R.P.6
Entelis, N.7
Tarassov, I.8
-
35
-
-
0035159226
-
The RNase P associated with HeLa cell mitochondria contains an essential RNA component identical in sequence to that of the nuclear RNase P
-
DOI 10.1128/MCB.21.2.548-561.2001
-
Puranam, R.S. and Attardi, G. (2001) The RNase P associated with HeLa cell mitochondria contains an essential RNA component identical in sequence to that of the nuclear RNase P. Mol. Cell Biol., 21, 548-561. (Pubitemid 32037374)
-
(2001)
Molecular and Cellular Biology
, vol.21
, Issue.2
, pp. 548-561
-
-
Puranam, R.S.1
Attardi, G.2
-
36
-
-
77955320529
-
PNPASE Regulates RNA Import into Mitochondria
-
Wang, G., Chen, H.W., Oktay, Y., Zhang, J., Allen, E.L., Smith, G.M., Fan, K.C., Hong, J.S., French, S.W., McCaffery, J.M. et al. (2010) PNPASE Regulates RNA Import into Mitochondria. Cell, 142, 456-467.
-
(2010)
Cell
, vol.142
, pp. 456-467
-
-
Wang, G.1
Chen, H.W.2
Oktay, Y.3
Zhang, J.4
Allen, E.L.5
Smith, G.M.6
Fan, K.C.7
Hong, J.S.8
French, S.W.9
McCaffery, J.M.10
-
37
-
-
77951153670
-
Selection of RNA aptamers imported into yeast and human mitochondria
-
Kolesnikova, O., Kazakova, H., Comte, C., Steinberg, S., Kamenski, P., Martin, R.P., Tarassov, I. and Entelis, N. (2010) Selection of RNA aptamers imported into yeast and human mitochondria. RNA, 16, 926-941.
-
(2010)
RNA
, vol.16
, pp. 926-941
-
-
Kolesnikova, O.1
Kazakova, H.2
Comte, C.3
Steinberg, S.4
Kamenski, P.5
Martin, R.P.6
Tarassov, I.7
Entelis, N.8
-
38
-
-
0034665704
-
Suppression of mutations in mitochondrial DNA by tRNAs imported from the cytoplasm
-
Kolesnikova, O.A., Entelis, N.S., Mireau, H., Fox, T.D., Martin, R.P. and Tarassov, I.A. (2000) Suppression of mutations in mitochondrial DNA by tRNAs imported from the cytoplasm. Science, 289, 1931-1933.
-
(2000)
Science
, vol.289
, pp. 1931-1933
-
-
Kolesnikova, O.A.1
Entelis, N.S.2
Mireau, H.3
Fox, T.D.4
Martin, R.P.5
Tarassov, I.A.6
-
39
-
-
9644264375
-
Nuclear DNA-encoded tRNAs targeted into mitochondria can rescue a mitochondrial DNA mutation associated with the MERRF syndrome in cultured human cells
-
DOI 10.1093/hmg/ddh267
-
Kolesnikova, O.A., Entelis, N.S., Jacquin-Becker, C., Goltzene, F., Chrzanowska-Lightowlers, Z.M., Lightowlers, R.N., Martin, R.P. and Tarassov, I. (2004) Nuclear DNA-encoded tRNAs targeted into mitochondria can rescue a mitochondrial DNA mutation associated with the MERRF syndrome in cultured human cells. Hum. Mol. Genet., 13, 2519-2534. (Pubitemid 39377855)
-
(2004)
Human Molecular Genetics
, vol.13
, Issue.20
, pp. 2519-2534
-
-
Kolesnikova, O.A.1
Entelis, N.S.2
Jacquin-Becker, C.3
Goltzene, F.4
Chrzanowska-Lightowlers, Z.M.5
Lightowlers, R.N.6
Martin, R.P.7
Tarassov, I.8
-
40
-
-
0033934844
-
Functional mitochondrial heterogeneity in heteroplasmic cells carrying the mitochondrial DNA mutation associated with the MELAS syndrome (mitochondrial encephalopathy, lactic acidosis, and strokelike episodes)
-
Bakker, A., Barthelemy, C., Frachon, P., Chateau, D., Sternberg, D., Mazat, J.P. and Lombes, A. (2000) Functional mitochondrial heterogeneity in heteroplasmic cells carrying the mitochondrial DNA mutation associated with the MELAS syndrome (mitochondrial encephalopathy, lactic acidosis, and strokelike episodes). Pediatr. Res., 48, 143-150. (Pubitemid 30497329)
-
(2000)
Pediatric Research
, vol.48
, Issue.2
, pp. 143-150
-
-
Bakker, A.1
Barthelemy, C.2
Frachon, P.3
Chateau, D.4
Sternberg, D.5
Mazat, J.P.6
Lombes, A.7
-
41
-
-
0024448458
-
Human cells lacking mtDNA: Repopulation with exogenous mitochondria by complementation
-
King, M.P. and Attardi, G. (1989) Human cells lacking mtDNA: repopulation with exogenous mitochondria by complementation. Science, 246, 500-503. (Pubitemid 19273941)
-
(1989)
Science
, vol.246
, Issue.4929
, pp. 500-503
-
-
King, M.P.1
Attardi, G.2
-
42
-
-
0032539720
-
Structural requirements of tRNALys for its import into yeast mitochondria
-
Entelis, N.S., Kieffer, S., Kolesnikova, O.A., Martin, R.P. and Tarassov, I.A. (1998) Structural requirements of tRNALys for its import into yeast mitochondria. Proc. Natl Acad. Sci. USA, 95, 2838-2843.
-
(1998)
Proc. Natl Acad. Sci. USA
, vol.95
, pp. 2838-2843
-
-
Entelis, N.S.1
Kieffer, S.2
Kolesnikova, O.A.3
Martin, R.P.4
Tarassov, I.A.5
-
43
-
-
33646033137
-
A lentiviral RNAi library for human and mouse genes applied to an arrayed viral high-content screen
-
Moffat, J., Grueneberg, D.A., Yang, X., Kim, S.Y., Kloepfer, A.M., Hinkle, G., Piqani, B., Eisenhaure, T.M., Luo, B., Grenier, J.K. et al. (2006) A lentiviral RNAi library for human and mouse genes applied to an arrayed viral high-content screen. Cell, 124, 1283-1298.
-
(2006)
Cell
, vol.124
, pp. 1283-1298
-
-
Moffat, J.1
Grueneberg, D.A.2
Yang, X.3
Kim, S.Y.4
Kloepfer, A.M.5
Hinkle, G.6
Piqani, B.7
Eisenhaure, T.M.8
Luo, B.9
Grenier, J.K.10
-
44
-
-
0037449126
-
Towards understanding human mitochondrial leucine aminoacylation identity
-
DOI 10.1016/S0022-2836(03)00373-5
-
Sohm, B., Frugier, M., Brule, H., Olszak, K., Przykorska, A. and Florentz, C. (2003) Towards understanding human mitochondrial leucine aminoacylation identity. J. Mol. Biol., 328, 995-1010. (Pubitemid 36506870)
-
(2003)
Journal of Molecular Biology
, vol.328
, Issue.5
, pp. 995-1010
-
-
Sohm, B.1
Frugier, M.2
Brule, H.3
Olszak, K.4
Przykorska, A.5
Florentz, C.6
-
45
-
-
2342527084
-
Leu(UUR) by its cognate leucyl-tRNA synthetase
-
DOI 10.1016/j.jmb.2004.03.066, PII S0022283604003717
-
Sohm, B., Sissler, M., Park, H., King, M.P. and Florentz, C. (2004) Recognition of human mitochondrial tRNALeu(UUR) by its cognate leucyl-tRNA synthetase. J. Mol. Biol., 339, 17-29. (Pubitemid 38569686)
-
(2004)
Journal of Molecular Biology
, vol.339
, Issue.1
, pp. 17-29
-
-
Sohm, B.1
Sissler, M.2
Park, H.3
King, M.P.4
Florentz, C.5
-
46
-
-
0034634569
-
The human lysyl-tRNA synthetase gene encodes both the cytoplasmic and mitochondrial enzymes by means of an unusual alternative splicing of the primary transcript
-
Tolkunova, E., Park, H., Xia, J., King, M.P. and Davidson, E. (2000) The human lysyl-tRNA synthetase gene encodes both the cytoplasmic and mitochondrial enzymes by means of an unusual alternative splicing of the primary transcript. J. Biol. Chem., 275, 35063-35069.
-
(2000)
J. Biol. Chem.
, vol.275
, pp. 35063-35069
-
-
Tolkunova, E.1
Park, H.2
Xia, J.3
King, M.P.4
Davidson, E.5
-
47
-
-
0026718556
-
The mitochondrial tRNA(Leu(UUR)) mutation in mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS): Genetic, biochemical, and morphological correlations in skeletal muscle
-
Moraes, C.T., Ricci, E., Bonilla, E., DiMauro, S. and Schon, E.A. (1992) The mitochondrial tRNA(Leu(UUR)) mutation in mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS): genetic, biochemical, and morphological correlations in skeletal muscle. Am. J. Hum. Genet., 50, 934-949.
-
(1992)
Am. J. Hum. Genet.
, vol.50
, pp. 934-949
-
-
Moraes, C.T.1
Ricci, E.2
Bonilla, E.3
Dimauro, S.4
Schon, E.A.5
-
48
-
-
0036363033
-
Import of nuclear encoded RNAs into yeast and human mitochondria: Experimental approaches and possible biomedical applications
-
Entelis, N., Kolesnikova, O., Kazakova, H., Brandina, I., Kamenski, P., Martin, R.P. and Tarassov, I. (2002) Import of nuclear encoded RNAs into yeast and human mitochondria: experimental approaches and possible biomedical applications. Genet. Eng., 24, 191-213.
-
(2002)
Genet. Eng.
, vol.24
, pp. 191-213
-
-
Entelis, N.1
Kolesnikova, O.2
Kazakova, H.3
Brandina, I.4
Kamenski, P.5
Martin, R.P.6
Tarassov, I.7
-
49
-
-
0026350896
-
Direct analysis of aminoacylation levels of tRNAs in Vivo: Application to studying recognition of Escherichia coli initiator tRNA mutants by glutaminyl-tRNA synthetase
-
Varshney, U., Lee, C.P. and RajBhandary, U.L. (1991) Direct analysis of aminoacylation levels of tRNAs in vivo. Application to studying recognition of Escherichia coli initiator tRNA mutants by glutaminyl-tRNA synthetase. J. Biol. Chem., 266, 24712-24718. (Pubitemid 21909000)
-
(1991)
Journal of Biological Chemistry
, vol.266
, Issue.36
, pp. 24712-24718
-
-
Varshney, U.1
Lee, C.-P.2
RajBhandary, U.L.3
-
50
-
-
0014949207
-
Cleavage of structural proteins during the assembly of the head of bacteriophage T4
-
Laemmli, U.K. (1970) Cleavage of structural proteins during the assembly of the head of bacteriophage T4. Nature, 227, 680-685.
-
(1970)
Nature
, vol.227
, pp. 680-685
-
-
Laemmli, U.K.1
-
51
-
-
0036838360
-
Targeting of tRNA into yeast and human mitochondria: The role of anticodon nucleotides
-
DOI 10.1016/S1567-7249(02)00013-2, PII S1567724902000132
-
Kolesnikova, O., Entelis, N., Kazakova, H., Brandina, I., Martin, R.P. and Tarassov, I. (2002) Targeting of tRNA into yeast and human mitochondria: the role of anticodon nucleotides. Mitochondrion, 2, 95-107. (Pubitemid 35214601)
-
(2002)
Mitochondrion
, vol.2
, Issue.1-2
, pp. 95-107
-
-
Kolesnikova, O.1
Entelis, N.2
Kazakova, H.3
Brandina, I.4
Martin, R.P.5
Tarassov, I.6
-
52
-
-
77951153670
-
Selection of RNA aptamers imported into yeast and human mitochondria
-
Kolesnikova, O., Kazakova, H., Comte, C., Steinberg, S., Kamenski, P., Martin, R.P., Tarassov, I. and Entelis, N. (2010) Selection of RNA aptamers imported into yeast and human mitochondria. RNA, 16, 926-941.
-
(2010)
RNA
, vol.16
, pp. 926-941
-
-
Kolesnikova, O.1
Kazakova, H.2
Comte, C.3
Steinberg, S.4
Kamenski, P.5
Martin, R.P.6
Tarassov, I.7
Entelis, N.8
-
53
-
-
0029017974
-
Mitochondrial import of a cytoplasmic lysine-tRNA in yeast is mediated by cooperation of cytoplasmic and mitochondrial lysyl-tRNA synthetases
-
Tarassov, I., Entelis, N. and Martin, R. (1995) Mitochondrial import of a cytoplasmic lysine-tRNA in yeast is mediated by cooperation of cytoplasmic and mitochondrial lysyl-tRNA synthetases. EMBO J., 14, 3461-3471.
-
(1995)
EMBO J.
, vol.14
, pp. 3461-3471
-
-
Tarassov, I.1
Entelis, N.2
Martin, R.3
-
54
-
-
0035797134
-
RNA delivery into mitochondria
-
DOI 10.1016/S0169-409X(01)00135-1, PII S0169409X01001351
-
Entelis, N.S., Kolesnikova, O.A., Martin, R.P. and Tarassov, I.A. (2001) RNA delivery into mitochondria. Adv. Drug Deliv. Rev., 49, 199-215. (Pubitemid 32493809)
-
(2001)
Advanced Drug Delivery Reviews
, vol.49
, Issue.1-2
, pp. 199-215
-
-
Entelis, N.S.1
Kolesnikova, O.A.2
Martin, R.P.3
Tarassov, I.A.4
-
55
-
-
0029923649
-
Mitochondrial import of a yeast cytoplasmic tRNA (Lys): Possible roles of aminoacylation and modified nucleosides in subcellular partitioning
-
Entelis, N.S., Krasheninnikov, I.A., Martin, R.P. and Tarassov, I.A. (1996) Mitochondrial import of a yeast cytoplasmic tRNA (Lys): possible roles of aminoacylation and modified nucleosides in subcellular partitioning. FEBS Lett., 384, 38-42.
-
(1996)
FEBS Lett.
, vol.384
, pp. 38-42
-
-
Entelis, N.S.1
Krasheninnikov, I.A.2
Martin, R.P.3
Tarassov, I.A.4
-
56
-
-
0034646656
-
Very rare complementation between mitochondria carrying different mitochondrial DNA mutations points to intrinsic genetic autonomy of the organelles in cultured human cells
-
DOI 10.1074/jbc.275.15.11207
-
Enriquez, J.A., Cabezas-Herrera, J., Bayona-Bafaluy, M.P. and Attardi, G. (2000) Very rare complementation between mitochondria carrying different mitochondrial DNA mutations points to intrinsic genetic autonomy of the organelles in cultured human cells. J. Biol. Chem., 275, 11207-11215. (Pubitemid 30212765)
-
(2000)
Journal of Biological Chemistry
, vol.275
, Issue.15
, pp. 11207-11215
-
-
Enriquez, J.A.1
Cabezas-Herrera, J.2
Bayona-Bafaluy, M.P.3
Attardi, G.4
|