Complex I biogenesis

Stable Identifier
R-HSA-6799198
Type
Pathway
Species
Homo sapiens
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Complex I (NADH:ubiquinone oxidoreductase or NADH dehydrogenase) utilises NADH formed from glycolysis and the TCA cycle to pump protons out of the mitochondrial matrix. It is the largest enzyme complex in the electron transport chain, containing 45 subunits. Seven subunits (ND1-6, ND4L) are encoded by mitochondrial DNA, the remainder encoded in the nucleus. The enzyme has a FMN prosthetic group and 8 Iron-Sulfur (Fe-S) clusters. The subunits are assembled together in a coordinated manner via preassembled subcomplexes to form the mature holoenzyme. The so-called "assembly factor" proteins, acting intrinsically or transiently, are required for constructing complex I although their exact roles in the biogenesis are not fully understood (Fernandez-Vizarra et al. 2009, Mckenzie & Ryan 2010, Mimaki et al. 2012, Andrews et al. 2013).

Literature References
PubMed ID Title Journal Year
20552642 Assembly factors of human mitochondrial complex I and their defects in disease

McKenzie, M, Ryan, MT

IUBMB Life 2010
21924235 Understanding mitochondrial complex I assembly in health and disease

Mimaki, M, Wang, X, McKenzie, M, Thorburn, DR, Ryan, MT

Biochim. Biophys. Acta 2012
24191001 Assembly factors for the membrane arm of human complex I

Andrews, B, Carroll, J, Ding, S, Fearnley, IM, Walker, JE

Proc. Natl. Acad. Sci. U.S.A. 2013
16760263 Assembly of mitochondrial cytochrome c-oxidase, a complicated and highly regulated cellular process

Fontanesi, F, Soto, IC, Horn, D, Barrientos, A

Am. J. Physiol., Cell Physiol. 2006
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