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
Compartment
mitochondrial inner membrane
ReviewStatus
5/5
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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. Furthermore, FAD-dependent oxidoreductase domain-containing protein 1 (FOXRED1) is required for binding of NDUFB6 in Complex I (Formosa et al., 2015). Defects in FOXRED1 lead to Complex I deficiency (Fassone et al., 2010). Sideroflexin 4 (SFXN4) is also required for the assembly of this module. It interacts with newly synthesized ND6. Defects in SFXN4 cause combined oxidative phosphorylation deficiency (Jackson et al., 2022).
Literature References
PubMed ID Title Journal Year
25678554 Characterization of mitochondrial FOXRED1 in the assembly of respiratory chain complex I

Formosa, LE, Mimaki, M, Frazier, AE, McKenzie, M, Stait, TL, Thorburn, DR, Stroud, DA, Ryan, MT

Hum Mol Genet 2015
32320651 Dissecting the Roles of Mitochondrial Complex I Intermediate Assembly Complex Factors in the Biogenesis of Complex I

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

Cell Rep 2020
20858599 FOXRED1, encoding an FAD-dependent oxidoreductase complex-I-specific molecular chaperone, is mutated in infantile-onset mitochondrial encephalopathy

Fassone, E, Duncan, AJ, Taanman, JW, Pagnamenta, AT, Sadowski, MI, Holand, T, Qasim, W, Rutland, P, Calvo, SE, Mootha, VK, Bitner-Glindzicz, M, Rahman, S

Hum Mol Genet 2010
35333655 Sideroflexin 4 is a complex I assembly factor that interacts with the MCIA complex and is required for the assembly of the ND2 module

Jackson, TD, Crameri, JJ, Muellner-Wong, L, Frazier, AE, Palmer, CS, Formosa, LE, Hock, DH, Fujihara, KM, Stait, T, Sharpe, AJ, Thorburn, DR, Ryan, MT, Stroud, DA, Stojanovski, D

Proc Natl Acad Sci U S A 2022
20552642 Assembly factors of human mitochondrial complex I and their defects in disease

McKenzie, M, Ryan, MT

IUBMB Life 2010
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
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COA1:MT-ND2, TMEM186:MT-ND3, MT-ND6, NDUFB6 bind the MCIA complex to form a 370kDa subcomplex (Bos taurus)
COA1:MT-ND2, TMEM186:MT-ND3, MT-ND6, NDUFB6 bind the MCIA complex to form a 370kDa subcomplex (Sus scrofa)
Authored
Jassal, B  (2015-09-22)
Reviewed
Meldal, BH  (2015-10-01)
Hill, DP  (2024-05-16)
Created
Jassal, B  (2015-09-22)
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