Human mitochondrial complex I assembly is mediated by NDUFAF1

FEBS Journal

Volumn 272, Issue 20, 2005, Pages 5317-5326

  Vogel, Rutger O ^a   Janssen, Rolf J R J ^a   Ugalde, Cristina ^a,b   Grovenstein, Melissa ^a   Huijbens, Richard J ^a   Visch, Henk Jan ^a   Van Den Heuvel, Lambert P ^a   Willems, Peter H ^a   Zeviani, Massimo ^c   Smeitink, Jan A M ^a   Nijtmans, Leo G J ^a  

^a RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

^b HOSPITAL UNIVERSITARIO 12 DE OCTUBRE   (Spain)

^c DEPARTMENT OF NEUROSURGERY   (Italy)

Author keywords

Assembly; Complex I; Mitochondria; NDUFAF1; Oxidative phosphorylation

Indexed keywords

MITOCHONDRIAL PROTEIN; PROTEIN NDUFAF1; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); UNCLASSIFIED DRUG;

ARTICLE; CELL STRAIN HEK293; CONTROLLED STUDY; GENE EXPRESSION; HUMAN; HUMAN CELL; MITOCHONDRION; PRIORITY JOURNAL; PROTEIN ASSEMBLY; PROTEIN STABILITY; RNA INTERFERENCE;

CELL FRACTIONATION; CELL LINE; DOXYCYCLINE; ELECTRON TRANSPORT COMPLEX I; ELECTRON TRANSPORT COMPLEX IV; HELA CELLS; HUMANS; MEMBRANE PROTEINS; MICROSCOPY, FLUORESCENCE; MITOCHONDRIA; MITOCHONDRIAL DISEASES; MITOCHONDRIAL PROTEINS; MUTATION; NAD(P)H DEHYDROGENASE (QUINONE); NADH DEHYDROGENASE; PROTEIN SUBUNITS; PROTEIN TRANSPORT; RNA, SMALL INTERFERING; TRANSFECTION;

NEUROSPORA CRASSA;

EID: 27144528747     PISSN: 1742464X     EISSN: 17424658     Source Type: Journal    
DOI: 10.1111/j.1742-4658.2005.04928.x     Document Type: Article

References (19)

1. Smeitink J, van den Heuvel L & DiMauro S (2001) The genetics and pathology of oxidative phosphorylation. Nat Rev Gene 2, 342-352.
2. Barrientos A, Barros MH, Valnot I, Rotig A, Rustin P & Tzagoloff A (2002) Cytochrome oxidase in health and disease. Gene 286, 53-63.
3. Janssen R, Smeitink J, Smeets R & van den Heuvel L (2002) CIA30 complex I assembly factor: a candidate for human complex I deficiency? Hum Genet 110, 264-270.
4. Grigorieff N (1999) Structure of the respiratory NADH: ubiquinone oxidoreductase (complex I). Curr Opin Struct Biol 9, 476-483.
5. Carroll J, Fearnley IM, Shannon RJ, Hirst J & Walker JE (2003) Analysis of the subunit composition of complex I from bovine heart mitochondria. Mol Cell Proteomics 2, 117-126.
6. Hirst J, Carroll J, Fearnley IM, Shannon RJ & Walker JE (2003) The nuclear encoded subunits of complex I from bovine heart mitochondria. Biochim Biophys Acta 1604, 135-150.
7. Vinogradov AD (2001) Respiratory complex I: structure, redox components, and possible mechanisms of energy transduction. Biochemistry 66, 1086-1097.
8. Cardol P, Matagne RF & Remacle C (2002) Impact of mutations affecting ND mitochondria-encoded subunits on the activity and assembly of complex I in Chlamydomonas. Implication for the structural organization of the enzyme. J Mol Biol 319, 1211-1221.
9. Stroh A, Anderka O, Pfeiffer K, Yagi T, Finel M, Ludwig B & Schagger H (2004) Assembly of respiratory complexes I, HI, and IV into NADH oxidase supercomplex stabilizes complex I in Paracoccus denitrificans. J Biol Chem 279, 5000-5007.
10. Yadava N, Houchens T, Potluri P & Scheffler IE (2004) Development and characterization of a conditional mitochondrial complex I assembly system. J Biol Chem 279, 12406-12413.
11. Videira A (1998) Complex I from the fungus Neurospora crassa. Biochim Biophys Acta 1364, 89-100.
12. Schulte U (2001) Biogenesis of respiratory complex I. J Bioenerg Biomembr 33, 205-212.
13. Antonicka H, Ogilvie I, Taivassalo T, Anitori RP, Haller RG, Vissing J, Kennaway NG & Shoubridge EA (2003) Identification and characterization of a common set of complex I assembly intermediates in mitochondria from patients with complex I deficiency. J Biol Chem 278, 43081-43088.
14. Ugalde C, Vogel R, Huijbens R, van den Heuvel B, Smeitink J & Nijtmans L (2004) Human mitochondrial complex I assembles through the combination of evolutionary conserved modules: a framework to interpret complex I deficiencies. Hum Mol Genet 13, 2461-2472.
15. Kuffner R, Rohr A, Schmiede A, Krull C & Schulte U (1998) Involvement of two novel chaperones in the assembly of mitochondrial NADH:Ubiquinone oxidoreductase (complex I). J Mol Biol 283, 409-417.
16. Duarte M & Videira A (2000) Respiratory chain complex I is essential for sexual development in Neurospora and binding of iron sulfur clusters are required for enzyme assembly. Genetics 156, 607-615.
17. Vahsen N, Cande C, Briere JJ, Benit P, Joza N, Larochette N, Mastroberardino PG, Pequignot MO, Casares N, Lazar V, Feraud O, Debili N, Wissing S, Engelhardt S, Madeo F, Piacentini M, Penninger JM, Schagger H, Rustin P & Kroemer G (2004) AIF deficiency compromises oxidative phosphorylation. EMBO J 23, 4679-4689.
18. Nijtmans LGJ, Henderson NS & Holt IJ (2002) Blue native electrophoresis to study mitochondrial and other protein complexes. Methods 26, 327-334.
19. Corona P, Antozzi C, Carrara F, D'Incerti L, Lamantea E, Tiranti V & Zeviani M (2001) A novel mtDNA mutation in the ND5 subunit of complex I in two MELAS patients. Ann Neurol 49, 106-110.