-
1
-
-
0032827410
-
Mitochondrial transport of cations: channels, exchangers, and permeability transition
-
Bernardi P. Mitochondrial transport of cations: channels, exchangers, and permeability transition. Physiol. Rev. 79 (1999) 1127-1155
-
(1999)
Physiol. Rev.
, vol.79
, pp. 1127-1155
-
-
Bernardi, P.1
-
3
-
-
4544378532
-
Mitochondrial fusion intermediates revealed in vitro
-
Meeusen S., McCaffery J.M., and Nunnari J. Mitochondrial fusion intermediates revealed in vitro. Science 305 (2004) 1747-1752
-
(2004)
Science
, vol.305
, pp. 1747-1752
-
-
Meeusen, S.1
McCaffery, J.M.2
Nunnari, J.3
-
4
-
-
0035474099
-
Nuclear genetic defects of oxidative phosphorylation
-
Shoubridge E.A. Nuclear genetic defects of oxidative phosphorylation. Hum. Mol. Genet. 10 (2001) 2277-2284
-
(2001)
Hum. Mol. Genet.
, vol.10
, pp. 2277-2284
-
-
Shoubridge, E.A.1
-
6
-
-
23844558266
-
A mitochondrial paradigm of metabolic and degenerative diseases, aging, and cancer: a dawn for evolutionary medicine
-
Wallace D.C. A mitochondrial paradigm of metabolic and degenerative diseases, aging, and cancer: a dawn for evolutionary medicine. Annu. Rev. Genet. 39 (2005) 359-407
-
(2005)
Annu. Rev. Genet.
, vol.39
, pp. 359-407
-
-
Wallace, D.C.1
-
7
-
-
33751574953
-
Bovine complex I is a component of 45 different subunits
-
Carrol J., Fearnley I.M., Skehel J.M., Shannon R.J., Hirst J., and Walker J.E. Bovine complex I is a component of 45 different subunits. J. Biol. Chem. 281 (2006) 32724-32727
-
(2006)
J. Biol. Chem.
, vol.281
, pp. 32724-32727
-
-
Carrol, J.1
Fearnley, I.M.2
Skehel, J.M.3
Shannon, R.J.4
Hirst, J.5
Walker, J.E.6
-
8
-
-
0942288076
-
Clinical heterogeneity in patients with mutations in the NDUFS4 gene of mitochondrial complex I
-
Budde S.M., van den Heuvel L.P., Smeets R.J., Skladal D., Mayr J.A., Boelen C., Petruzzella V., Papa S., and Smeitink J.A.M. Clinical heterogeneity in patients with mutations in the NDUFS4 gene of mitochondrial complex I. J. Inherit. Metab. Dis. 26 (2003) 813-815
-
(2003)
J. Inherit. Metab. Dis.
, vol.26
, pp. 813-815
-
-
Budde, S.M.1
van den Heuvel, L.P.2
Smeets, R.J.3
Skladal, D.4
Mayr, J.A.5
Boelen, C.6
Petruzzella, V.7
Papa, S.8
Smeitink, J.A.M.9
-
9
-
-
9144221957
-
Mutant NDUFS3 subunit of mitochondrial complex I causes Leigh syndrome
-
Benit P., Slama A., Cartault F., Giurgea I., Chretien D., Lebon S., Marsac C., Munnich A., Rotig A., and Rustin P. Mutant NDUFS3 subunit of mitochondrial complex I causes Leigh syndrome. J. Med. Genet. 41 (2004) 14-17
-
(2004)
J. Med. Genet.
, vol.41
, pp. 14-17
-
-
Benit, P.1
Slama, A.2
Cartault, F.3
Giurgea, I.4
Chretien, D.5
Lebon, S.6
Marsac, C.7
Munnich, A.8
Rotig, A.9
Rustin, P.10
-
11
-
-
8844244960
-
NDUFS6 mutations are a novel cause of lethal neonatal mitochondrial complex I deficiency
-
Kirby D.M., Salemi R., Sugiana C., Ohtake A., Parry L., Bell K.M., Kirk E.P., Boneh A., Taylor R.W., Dahl H.H., Ryan M.T., and Thorburn D.R. NDUFS6 mutations are a novel cause of lethal neonatal mitochondrial complex I deficiency. J. Clin. Invest. 114 (2004) 837-845
-
(2004)
J. Clin. Invest.
, vol.114
, pp. 837-845
-
-
Kirby, D.M.1
Salemi, R.2
Sugiana, C.3
Ohtake, A.4
Parry, L.5
Bell, K.M.6
Kirk, E.P.7
Boneh, A.8
Taylor, R.W.9
Dahl, H.H.10
Ryan, M.T.11
Thorburn, D.R.12
-
12
-
-
26444488636
-
A molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy
-
Ogilvie I., Kennaway N.G., and Shoubridge E.A. A molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy. J. Clin. Invest. 115 (2005) 2784-2792
-
(2005)
J. Clin. Invest.
, vol.115
, pp. 2784-2792
-
-
Ogilvie, I.1
Kennaway, N.G.2
Shoubridge, E.A.3
-
13
-
-
1642382090
-
Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency
-
Ugalde C., Janssen R.J., van den Heuvel L.W., Smeitink J.A.M., and Nijtmans L.G. Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency. Hum. Mol. Genet. 13 (2004) 659-667
-
(2004)
Hum. Mol. Genet.
, vol.13
, pp. 659-667
-
-
Ugalde, C.1
Janssen, R.J.2
van den Heuvel, L.W.3
Smeitink, J.A.M.4
Nijtmans, L.G.5
-
14
-
-
33744515907
-
Dysfunctions of cellular oxidative metabolism in patients with mutations in the NDUFS1 and NDUFS4 genes of complex I
-
Iuso A., Scacco S., Piccoli C., Bellomo F., Petruzzella V., Trentadue R., Minuto M., Ripoli M., Capitanio N., Zeviani M., and Papa S. Dysfunctions of cellular oxidative metabolism in patients with mutations in the NDUFS1 and NDUFS4 genes of complex I. J. Biol. Chem. 281 (2006) 10374-10380
-
(2006)
J. Biol. Chem.
, vol.281
, pp. 10374-10380
-
-
Iuso, A.1
Scacco, S.2
Piccoli, C.3
Bellomo, F.4
Petruzzella, V.5
Trentadue, R.6
Minuto, M.7
Ripoli, M.8
Capitanio, N.9
Zeviani, M.10
Papa, S.11
-
15
-
-
9644283053
-
Application of the yeast Yarrowia lipolytica as a model to analyse human pathogenic mutations in mitochondrial complex I (NADH:ubiquinone oxidoreductase)
-
Kerscher S., Grgic L., Garofano A., and Brandt U. Application of the yeast Yarrowia lipolytica as a model to analyse human pathogenic mutations in mitochondrial complex I (NADH:ubiquinone oxidoreductase). Biochim. Biophys. Acta 1659 (2004) 197-205
-
(2004)
Biochim. Biophys. Acta
, vol.1659
, pp. 197-205
-
-
Kerscher, S.1
Grgic, L.2
Garofano, A.3
Brandt, U.4
-
16
-
-
25444446126
-
Mitochondrial network complexity and pathological decrease in complex I activity are tightly correlated in isolated human complex I deficiency
-
Koopman W.J.H., Visch H.J., Verkaart S., van den Heuvel L.W., Smeitink J.A.M., and Willems P.H.G.M. Mitochondrial network complexity and pathological decrease in complex I activity are tightly correlated in isolated human complex I deficiency. Am. J. Physiol., Cell Physiol. 289 (2005) C881-C890
-
(2005)
Am. J. Physiol., Cell Physiol.
, vol.289
-
-
Koopman, W.J.H.1
Visch, H.J.2
Verkaart, S.3
van den Heuvel, L.W.4
Smeitink, J.A.M.5
Willems, P.H.G.M.6
-
17
-
-
4644326685
-
2+ handling in human complex I deficiency
-
2+ handling in human complex I deficiency. J. Biol. Chem. 279 (2004) 40328-40336
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 40328-40336
-
-
Visch, H.J.1
Rutter, G.A.2
Koopman, W.J.H.3
Koenderink, J.B.4
Verkaart, S.5
de Groot, T.6
Varadi, A.7
Mitchell, K.J.8
van den Heuvel, L.W.9
Smeitink, J.A.M.10
Willems, P.H.G.M.11
-
18
-
-
29644439802
-
Decreased agonist-stimulated mitochondrial ATP production caused by a pathological reduction in endoplasmic reticulum calcium content in human complex I deficiency
-
Visch H.J., Koopman W.J.H., Leusink A., van Emst-de Vries S.E., van den Heuvel L.W., Willems P.H.G.M., and Smeitink J.A.M. Decreased agonist-stimulated mitochondrial ATP production caused by a pathological reduction in endoplasmic reticulum calcium content in human complex I deficiency. Biochim. Biophys. Acta 1762 (2005) 115-123
-
(2005)
Biochim. Biophys. Acta
, vol.1762
, pp. 115-123
-
-
Visch, H.J.1
Koopman, W.J.H.2
Leusink, A.3
van Emst-de Vries, S.E.4
van den Heuvel, L.W.5
Willems, P.H.G.M.6
Smeitink, J.A.M.7
-
19
-
-
1842338036
-
Excessive formation of hydroxyl radicals and aldehydic lipid peroxidation products in cultured skin fibroblasts from patients with complex I deficiency
-
Luo X., Pitkänen S., Kassovska-Bratinova S., Robinson B.H., and Lehotay D.C. Excessive formation of hydroxyl radicals and aldehydic lipid peroxidation products in cultured skin fibroblasts from patients with complex I deficiency. J. Clin. Invest. 99 (1997) 2877-2882
-
(1997)
J. Clin. Invest.
, vol.99
, pp. 2877-2882
-
-
Luo, X.1
Pitkänen, S.2
Kassovska-Bratinova, S.3
Robinson, B.H.4
Lehotay, D.C.5
-
20
-
-
0030056515
-
Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase
-
Pitkänen S., and Robinson B.H. Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase. J. Clin. Invest. 98 (1996) 345-351
-
(1996)
J. Clin. Invest.
, vol.98
, pp. 345-351
-
-
Pitkänen, S.1
Robinson, B.H.2
-
21
-
-
29344464774
-
Mitochondrial dysfunction in cardiac ischemia-reperfusion injury: ROS from complex I, without inhibition
-
Tompkins A.J., Burwell L.S., Digerness S.B., Zaragosa C., Holman W.L., and Brookes P.S. Mitochondrial dysfunction in cardiac ischemia-reperfusion injury: ROS from complex I, without inhibition. Biochim. Biophys. Acta 1762 (2006) 223-231
-
(2006)
Biochim. Biophys. Acta
, vol.1762
, pp. 223-231
-
-
Tompkins, A.J.1
Burwell, L.S.2
Digerness, S.B.3
Zaragosa, C.4
Holman, W.L.5
Brookes, P.S.6
-
22
-
-
33845950116
-
Simultaneous quantification of oxidative stress and cell spreading using 5-(and -6)-chloromethyl-2′,7′-dichlorofluorescein
-
Koopman W.J.H., Verkaart S., van Emst-de Vries S.E., Grefte S., Smeitink J.A.M., and Willems P.H.G.M. Simultaneous quantification of oxidative stress and cell spreading using 5-(and -6)-chloromethyl-2′,7′-dichlorofluorescein. Cytometry A 69 (2006) 1184-1192
-
(2006)
Cytometry A
, vol.69
, pp. 1184-1192
-
-
Koopman, W.J.H.1
Verkaart, S.2
van Emst-de Vries, S.E.3
Grefte, S.4
Smeitink, J.A.M.5
Willems, P.H.G.M.6
-
23
-
-
33847031295
-
Superoxide production is inversely related to complex I activity in inherited complex I deficiency
-
Verkaart S., Koopman W.J.H., van Emst-de Vries S.E., Nijtmans L.G., van den Heuvel L.W., Smeitink J.A.M., and Willems P.H.G.M. Superoxide production is inversely related to complex I activity in inherited complex I deficiency. Biochim. Biophys. Acta 1772 (2007) 373-381
-
(2007)
Biochim. Biophys. Acta
, vol.1772
, pp. 373-381
-
-
Verkaart, S.1
Koopman, W.J.H.2
van Emst-de Vries, S.E.3
Nijtmans, L.G.4
van den Heuvel, L.W.5
Smeitink, J.A.M.6
Willems, P.H.G.M.7
-
24
-
-
34548174026
-
Mitochondrial and cytosolic thiol redox state are not detectably altered in isolated human NADH:ubiquinone oxidoreductase deficiency
-
Verkaart S., Koopman W.J.H., Cheek J., van Emst-de Vries S.E., van den Heuvel L.W., Smeitink J.A.M., and Willems P.H.G.M. Mitochondrial and cytosolic thiol redox state are not detectably altered in isolated human NADH:ubiquinone oxidoreductase deficiency. Biochim. Biophys. Acta 1772 (2007) 1041-1051
-
(2007)
Biochim. Biophys. Acta
, vol.1772
, pp. 1041-1051
-
-
Verkaart, S.1
Koopman, W.J.H.2
Cheek, J.3
van Emst-de Vries, S.E.4
van den Heuvel, L.W.5
Smeitink, J.A.M.6
Willems, P.H.G.M.7
-
25
-
-
1342264360
-
Oxidatively damaged proteins of heart mitochondrial electron transport complexes
-
Choksi K.B., Boylston W.H., Rabek J.P., Widger W.R., and Papaconstantinou J. Oxidatively damaged proteins of heart mitochondrial electron transport complexes. Biochim. Biophys. Acta 1688 (2004) 95-101
-
(2004)
Biochim. Biophys. Acta
, vol.1688
, pp. 95-101
-
-
Choksi, K.B.1
Boylston, W.H.2
Rabek, J.P.3
Widger, W.R.4
Papaconstantinou, J.5
-
26
-
-
27844605495
-
Superoxide generation from mitochondrial NADH dehydrogenase induces self inactivation with specific protein radical formation
-
Chen Y.R., Chen C.W., Zhang L., Green-church K.B., and Zweier J.L. Superoxide generation from mitochondrial NADH dehydrogenase induces self inactivation with specific protein radical formation. J. Biol. Chem. 280 (2005) 37339-37348
-
(2005)
J. Biol. Chem.
, vol.280
, pp. 37339-37348
-
-
Chen, Y.R.1
Chen, C.W.2
Zhang, L.3
Green-church, K.B.4
Zweier, J.L.5
-
27
-
-
27744508427
-
Rapid rates of newly synthesized mitochondrial protein degradation are significantly effected by the generation of mitochondrial free radicals
-
Basoah A., Matthews P.M., and Morten K.J. Rapid rates of newly synthesized mitochondrial protein degradation are significantly effected by the generation of mitochondrial free radicals. FEBS Lett. 579 (2005) 6511-6517
-
(2005)
FEBS Lett.
, vol.579
, pp. 6511-6517
-
-
Basoah, A.1
Matthews, P.M.2
Morten, K.J.3
-
28
-
-
0942298545
-
Endogenous mitochondrial oxidative stress: neurodegeneration, proteomic analysis, specific respiratory chain defects, and efficacious antioxidant therapy in superoxide dismutase 2 null mice
-
Hinerfeld D., Traini M.D., Weinberger R.P., Cochran B., Doctrow S.R., Harry J., and Melov S. Endogenous mitochondrial oxidative stress: neurodegeneration, proteomic analysis, specific respiratory chain defects, and efficacious antioxidant therapy in superoxide dismutase 2 null mice. J. Neurochem. 88 (2004) 657-667
-
(2004)
J. Neurochem.
, vol.88
, pp. 657-667
-
-
Hinerfeld, D.1
Traini, M.D.2
Weinberger, R.P.3
Cochran, B.4
Doctrow, S.R.5
Harry, J.6
Melov, S.7
-
29
-
-
1942453308
-
The mtDNA T8993G (NARP) mutation results in an impairment of oxidative phosphorylation that can be improved by antioxidants
-
Mattiazzi M., Vijayvergiya C., Gajewski C.D., DeVivo D.C., Lenaz G., Wiedmann M., and Manfredi G. The mtDNA T8993G (NARP) mutation results in an impairment of oxidative phosphorylation that can be improved by antioxidants. Hum. Mol. Genet. 13 (2004) 869-879
-
(2004)
Hum. Mol. Genet.
, vol.13
, pp. 869-879
-
-
Mattiazzi, M.1
Vijayvergiya, C.2
Gajewski, C.D.3
DeVivo, D.C.4
Lenaz, G.5
Wiedmann, M.6
Manfredi, G.7
-
30
-
-
27144436412
-
Vitamin E at high doses improves survival, neurological performance and brain mitochondrial function in aging male mice
-
Navarro A., Gomez C., Sanchez-Pino M.J., Gonzalez H., Bandez M.J., Boveris A.D., and Boveris A. Vitamin E at high doses improves survival, neurological performance and brain mitochondrial function in aging male mice. Am. J. Physiol., Regul. Integr. Comp. Physiol. 289 (2005) R1392-R1399
-
(2005)
Am. J. Physiol., Regul. Integr. Comp. Physiol.
, vol.289
-
-
Navarro, A.1
Gomez, C.2
Sanchez-Pino, M.J.3
Gonzalez, H.4
Bandez, M.J.5
Boveris, A.D.6
Boveris, A.7
-
31
-
-
4344575973
-
Effect of high-dose vitamins, coenzyme Q and high-fat diet in paediatric patients with mitochondrial diseases
-
Panetta J., Smith L.J., and Boneh A. Effect of high-dose vitamins, coenzyme Q and high-fat diet in paediatric patients with mitochondrial diseases. J. Inherit. Metab. Dis. 27 (2004) 487-498
-
(2004)
J. Inherit. Metab. Dis.
, vol.27
, pp. 487-498
-
-
Panetta, J.1
Smith, L.J.2
Boneh, A.3
-
33
-
-
0036024975
-
Blue Native electrophoresis to study mitochondrial and other protein complexes
-
Nijtmans L.G., Henderson N.S., and Holt I.J. Blue Native electrophoresis to study mitochondrial and other protein complexes. Methods 26 (2002) 327-334
-
(2002)
Methods
, vol.26
, pp. 327-334
-
-
Nijtmans, L.G.1
Henderson, N.S.2
Holt, I.J.3
-
34
-
-
19544369483
-
Human mitochondrial complex I assembles through the combination of evolutionary conserved modules: a framework to interpret complex I deficiencies
-
Ugalde C., Vogel R., Huijbens R., van den Heuvel L.W., Smeitink J.A.M., and Nijtmans L.G. Human mitochondrial complex I assembles through the combination of evolutionary conserved modules: a framework to interpret complex I deficiencies. Hum. Mol. Genet. 13 (2004) 2461-2472
-
(2004)
Hum. Mol. Genet.
, vol.13
, pp. 2461-2472
-
-
Ugalde, C.1
Vogel, R.2
Huijbens, R.3
van den Heuvel, L.W.4
Smeitink, J.A.M.5
Nijtmans, L.G.6
-
35
-
-
0035937748
-
Human complex I defects can be resolved by monoclonal antibody analysis into distinct subunit assembly patterns
-
Triepels R.H., Hanson B.J., van den Heuvel L.W., Sundell L., Marusich M.F., Smeitink J.A.M., and Capaldi R.A. Human complex I defects can be resolved by monoclonal antibody analysis into distinct subunit assembly patterns. J. Biol. Chem. 276 (2001) 8892-8897
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 8892-8897
-
-
Triepels, R.H.1
Hanson, B.J.2
van den Heuvel, L.W.3
Sundell, L.4
Marusich, M.F.5
Smeitink, J.A.M.6
Capaldi, R.A.7
-
36
-
-
34547128370
-
Human NADH:ubiquinone oxidoreductase deficiency: radical changes in mitochondrial morphology?
-
Koopman W.J.H., Verkaart S., Visch H.J., Nijtmans L.G.J., Smeitink J.A.M., and Willems P.H.G.M. Human NADH:ubiquinone oxidoreductase deficiency: radical changes in mitochondrial morphology?. Am. J. Physiol., Cell Physiol. 293 (2007) C22-C29
-
(2007)
Am. J. Physiol., Cell Physiol.
, vol.293
-
-
Koopman, W.J.H.1
Verkaart, S.2
Visch, H.J.3
Nijtmans, L.G.J.4
Smeitink, J.A.M.5
Willems, P.H.G.M.6
-
37
-
-
0034663704
-
Application of the obligate aerobic yeast Yarrowia lipolytica as a eucaryotic model to analyse Leigh Syndrome mutations in the complex I core subunits PSST and TYKY
-
Ahlers P., Garofano A., Kerscher S., and Brandt U. Application of the obligate aerobic yeast Yarrowia lipolytica as a eucaryotic model to analyse Leigh Syndrome mutations in the complex I core subunits PSST and TYKY. Biochim. Biophys. Acta 1459 (2000) 258-265
-
(2000)
Biochim. Biophys. Acta
, vol.1459
, pp. 258-265
-
-
Ahlers, P.1
Garofano, A.2
Kerscher, S.3
Brandt, U.4
-
38
-
-
22444434735
-
Neurospora strains harboring mitochondrial disease-associated mutations in iron-sulfur subunits of complex I
-
Duarte M., Schulte U., Ushakova A.V., and Videira A. Neurospora strains harboring mitochondrial disease-associated mutations in iron-sulfur subunits of complex I. Genetics 171 (2005) 91-99
-
(2005)
Genetics
, vol.171
, pp. 91-99
-
-
Duarte, M.1
Schulte, U.2
Ushakova, A.V.3
Videira, A.4
-
39
-
-
33746329868
-
Energy converting NADH:quinone oxidoreductase (complex I)
-
Brandt U. Energy converting NADH:quinone oxidoreductase (complex I). Annu. Rev. Biochem. 75 (2006) 69-92
-
(2006)
Annu. Rev. Biochem.
, vol.75
, pp. 69-92
-
-
Brandt, U.1
-
40
-
-
33751072935
-
Bioenergetics and the formation of mitochondrial reactive oxygen species
-
Adam-Vizi V., and Chinopoulos C. Bioenergetics and the formation of mitochondrial reactive oxygen species. Trends Pharmacol. Sci. 27 (2006) 639-645
-
(2006)
Trends Pharmacol. Sci.
, vol.27
, pp. 639-645
-
-
Adam-Vizi, V.1
Chinopoulos, C.2
-
41
-
-
27144537231
-
Prooxidant and antioxidant activity of vitamin E analogues and troglitazone
-
Tafazoli S., Wright J.S., and O'Brien P.J. Prooxidant and antioxidant activity of vitamin E analogues and troglitazone. Chem. Res. Toxicol. 18 (2005) 1567-1574
-
(2005)
Chem. Res. Toxicol.
, vol.18
, pp. 1567-1574
-
-
Tafazoli, S.1
Wright, J.S.2
O'Brien, P.J.3
-
42
-
-
0036871750
-
Regulation of cell signaling by vitamin E
-
Rimbach G., Minihane A.M., Majewicz J., Fischer A., Pallauf J., Virgli E., and Weinberg P.D. Regulation of cell signaling by vitamin E. Proc. Nutr. Soc. 61 (2002) 415-425
-
(2002)
Proc. Nutr. Soc.
, vol.61
, pp. 415-425
-
-
Rimbach, G.1
Minihane, A.M.2
Majewicz, J.3
Fischer, A.4
Pallauf, J.5
Virgli, E.6
Weinberg, P.D.7
-
43
-
-
0037029129
-
Reactive oxygen species affect mitochondrial electron transport complex I activity through oxidative cardiolipin damage
-
Paradies G., Petrosillo G., Pistolese M., and Ruggiero F.M. Reactive oxygen species affect mitochondrial electron transport complex I activity through oxidative cardiolipin damage. Gene 286 (2002) 135-141
-
(2002)
Gene
, vol.286
, pp. 135-141
-
-
Paradies, G.1
Petrosillo, G.2
Pistolese, M.3
Ruggiero, F.M.4
-
44
-
-
33644850965
-
Depletion of cardiolipin and cytochrome c during ischemia increases hydrogen peroxide production from the electron transport chain
-
Chen Q., and Lesnefsky E.J. Depletion of cardiolipin and cytochrome c during ischemia increases hydrogen peroxide production from the electron transport chain. Free Rad. Biol. Med. 40 (2006) 976-982
-
(2006)
Free Rad. Biol. Med.
, vol.40
, pp. 976-982
-
-
Chen, Q.1
Lesnefsky, E.J.2
-
45
-
-
20444461625
-
The Charcot-Marie-Tooth type 2A gene product, Mfn2, up-regulates fuel oxidation through expression of OXPHOS system
-
Pich S., Bach D., Briones P., Liesa M., Camps M., Testar X., Palacin M., and Zorzano A. The Charcot-Marie-Tooth type 2A gene product, Mfn2, up-regulates fuel oxidation through expression of OXPHOS system. Hum. Mol. Genet. 14 (2005) 1405-1415
-
(2005)
Hum. Mol. Genet.
, vol.14
, pp. 1405-1415
-
-
Pich, S.1
Bach, D.2
Briones, P.3
Liesa, M.4
Camps, M.5
Testar, X.6
Palacin, M.7
Zorzano, A.8
-
46
-
-
0035132188
-
Mutations in the complex I NDUFS2 gene of patient with cardiomyopathy and encephalomyopathy
-
Loeffen J.L., Elpeleg O., Smeitink J.A.M., Smeets R.J., Stöckler-Ipsiroglu S., Mandel H., Sengers R., Trijbels F., and van den Heuvel L.P. Mutations in the complex I NDUFS2 gene of patient with cardiomyopathy and encephalomyopathy. Ann. Neurol. 49 (2001) 195-201
-
(2001)
Ann. Neurol.
, vol.49
, pp. 195-201
-
-
Loeffen, J.L.1
Elpeleg, O.2
Smeitink, J.A.M.3
Smeets, R.J.4
Stöckler-Ipsiroglu, S.5
Mandel, H.6
Sengers, R.7
Trijbels, F.8
van den Heuvel, L.P.9
-
47
-
-
0032977683
-
Mutant NDUFV1 subunit of mitochondrial complex I causes leukodystrophy and myoclonic epilepsy
-
Schuelke M., Smeitink J.A.M., Mariman E., Loeffen J., Plecko B., Trijbels F., Stockler-Ipsiroglu S., and van den Heuvel L.W. Mutant NDUFV1 subunit of mitochondrial complex I causes leukodystrophy and myoclonic epilepsy. Nat. Genet. 21 (1999) 260-261
-
(1999)
Nat. Genet.
, vol.21
, pp. 260-261
-
-
Schuelke, M.1
Smeitink, J.A.M.2
Mariman, E.3
Loeffen, J.4
Plecko, B.5
Trijbels, F.6
Stockler-Ipsiroglu, S.7
van den Heuvel, L.W.8
-
48
-
-
0033050180
-
Leigh syndrome associated with a mutation in the NDUFS7 (PSST) nuclear encoded subunit of complex I
-
Triepels R.H., van den Heuvel L.P., Loeffen J.L., Buskens C.A., Smeets R.J., Rubio Gozalbo M.E., Mariman E.C., Wijburg F.A., Barth P.G., Trijbels J.M., and Smeitink J.A.M. Leigh syndrome associated with a mutation in the NDUFS7 (PSST) nuclear encoded subunit of complex I. Ann. Neurol. 45 (1999) 787-790
-
(1999)
Ann. Neurol.
, vol.45
, pp. 787-790
-
-
Triepels, R.H.1
van den Heuvel, L.P.2
Loeffen, J.L.3
Buskens, C.A.4
Smeets, R.J.5
Rubio Gozalbo, M.E.6
Mariman, E.C.7
Wijburg, F.A.8
Barth, P.G.9
Trijbels, J.M.10
Smeitink, J.A.M.11
|