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

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

Species

Homo sapiens

Compartment

mitochondrial inner membrane

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

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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
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
20552642	Assembly factors of human mitochondrial complex I and their defects in disease Ryan, MT, McKenzie, M	IUBMB Life	2010
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