Reactome | MT-CO3, COX6A,B,7A and NDUFA4 bind to holo-MT-CO1,2 complex

MT-CO3, COX6A,B,7A and NDUFA4 bind to holo-MT-CO1,2 complex

Stable Identifier

R-HSA-9865663

Type

Reaction [omitted]

Species

Homo sapiens

Compartment

mitochondrial inner membrane

ReviewStatus

5/5

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MT-CO3, COX6A,B,7A and NDUFA4 bind to holo-MT-CO1,2 complex

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The final assembly of Complex IV consists of binding of the MT-CO3, COX6A, COX6B, COX7A, and NDUFA4 subunits to the holo-MT-CO1, MT-CO2 complex (reviewed by, e.g., Timón-Gómez et al., 2018). Both HIGD1A and HIGD2A act as positive regulators of the final assembly (Vukotic et al., 2012; Hock et al., 2020; Timón-Gómez et al., 2020a; reviewed in Timón-Gómez et al., 2020b). Recent data also points to RAB5IF as an assembly cofactor (Moutaoufik et al., 2019). Patients with NDUFA4 mutations display COX deficiencies (Pitceathly et al., 2013).

Literature References

PubMed ID	Title	Journal	Year
32375044	Distinct Roles of Mitochondrial HIGD1A and HIGD2A in Respiratory Complex and Supercomplex Biogenesis Ugalde, C, Timón-Gómez, A, Garlich, J, Barrientos, A, Stuart, RA	Cell Rep	2020
22342701	Rcf1 mediates cytochrome oxidase assembly and respirasome formation, revealing heterogeneity of the enzyme complex Wiese, S, Vögtle, FN, Meyer, HE, Jakobs, S, Jans, DC, Vukotic, M, Katschinski, DM, Deckers, M, Warscheid, B, Rehling, P, Zieseniss, A, Meisinger, C, Oeljeklaus, S	Cell Metab.	2012
23746447	NDUFA4 mutations underlie dysfunction of a cytochrome c oxidase subunit linked to human neurological disease Hurles, ME, Rahman, S, Taanman, JW, Woodward, CE, Sweeney, MG, Foley, AR, Pitceathly, RD, Sailer, A, Houlden, H, Muntoni, F, Stalker, J, Wedatilake, Y, Polke, JM, Hanna, MG, Cirak, S, Hargreaves, I	Cell Rep	2013
31536960	Rewiring of the Human Mitochondrial Interactome during Neuronal Reprogramming Reveals Regulators of the Respirasome and Neurogenesis Zhang, Q, Gagarinova, A, Moutaoufik, MT, Musso, G, Malty, R, Okazaki, Y, Zilocchi, M, Phanse, S, Bader, GD, Scott, NE, Rajput, A, Hoell, L, Foster, LJ, Vlasblom, J, Broderick, K, Gagarinova, M, Lamantea, E, Amin, S, Minic, Z, Jessulat, M, Goebels, F, Stockwell, J, Prasad, B, Aoki, H, Babu, M, Garavaglia, B, Murayama, K, Cayabyab, FS	iScience	2019
28870773	Mitochondrial cytochrome c oxidase biogenesis: Recent developments Timón-Gómez, A, Nývltová, E, Barrientos, A, Abriata, LA, Vila, AJ, Hosler, J	Semin Cell Dev Biol	2018
32317297	HIGD2A is Required for Assembly of the COX3 Module of Human Mitochondrial Complex IV Stroud, DA, Compton, AG, Ang, CS, Hock, DH, Ryan, MT, Thorburn, DR, Reljic, B, Muellner-Wong, L, Mountford, HS	Mol Cell Proteomics	2020
33291261	HIGD-Driven Regulation of Cytochrome c Oxidase Biogenesis and Function Timón-Gómez, A, Barrientos, A, Fontanesi, F, Bartley-Dier, EL	Cells	2020