COA1:MT-ND2, TMEM186:MT-ND3, MT-ND6, NDUFB6 bind the MCIA complex to form a 370kDa subcomplex

Stable Identifier
R-HSA-6799199
Type
Reaction [binding]
Species
Homo sapiens
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Membrane arm subunits COA1:MT-ND2, TMEM186:MT-ND3, MT-ND6 and NDUFB6 associate with the assembly factors TMEM126B, NDUFAF1, ECSIT and ACAD9 (which form the MCIA complex) forming a 370kDa subcomplex (Mckenzie & Ryan 2010, Andrews et al. 2013). Formosa et al. identified TMEM186 and COA1 as components of the MCIA complex with loss of either resulting in MCIA complex defects and reduced complex I assembly (Formosa et al. 2020). Transmembrane protein 186 (TMEM186) interacts strongly with ND3 while cytochrome c oxidase assembly factor 1 homolog (COA1) interacts with ND2.

Literature References
PubMed ID Title Journal Year
24191001 Assembly factors for the membrane arm of human complex I

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

Proc. Natl. Acad. Sci. U.S.A. 2013
20552642 Assembly factors of human mitochondrial complex I and their defects in disease

Ryan, MT, McKenzie, M

IUBMB Life 2010
32320651 Dissecting the Roles of Mitochondrial Complex I Intermediate Assembly Complex Factors in the Biogenesis of Complex I

Stroud, DA, Beilharz, TH, Formosa, LE, Stojanovski, D, Ryan, MT, Jackson, TD, Reljic, B, Muellner-Wong, L, Sharpe, AJ, Lazarou, M

Cell Rep 2020
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