Reactome | Complex III assembly

Complex III assembly

Stable Identifier

R-HSA-9865881

DOI

10.3180/R-HSA-9865881.1

Type

Pathway

Species

Homo sapiens

Compartment

mitochondrial inner membrane, mitochondrial matrix

ReviewStatus

5/5

Locations in the PathwayBrowser

Expand all

General

SBML | | PDF

SVG | | PPTX | SBGN

Click the image above or here to open this pathway in the Pathway Browser

Assembly of the cytochrome c (cytochrome bc1) reductase (Complex III) was mainly investigated in yeast. The process in humans is considered to recapitulate the process in the yeast system based on the high similarity in structure and composition of the yeast and human complexes. Human Complex III probably consists of two identical sub-complexes, each containing at least 11 subunits: the catalytic core containing cytochrome b (MT-CYTB), cytochrome c1 (CYC1), and the Rieske protein (UQCRFS1), as well as the additional subunits UQCRC1, UQCRC2, UQCRC10, UQCRC11, UQCRCB, UQCRCH, UQCRCQ (Guo et al., 2017; Dennerlein et al., 2021; reviewed in Ndi et al., 2018; Signes & Fernandez-Vizarra, 2018). While complex I, III, and IV form a supercomplex, there is no evidence of any physiological advantage of this configuration (reviewed in Kohler et al., 2023; Brischigliaro et al., 2023). Mutations in nuclear genes coding for subunits of Complex III, as well as assembly factors, can cause complex III deficiency (MC3D; reviewed in Fernández-Vizarra & Zeviani, 2015).

Literature References

PubMed ID	Title	Journal	Year
30030361	Assembly of mammalian oxidative phosphorylation complexes I-V and supercomplexes Signes, A, Fernandez-Vizarra, E	Essays Biochem	2018
25914718	Nuclear gene mutations as the cause of mitochondrial complex III deficiency Fernandez-Vizarra, E, Zeviani, M	Front Genet	2015
29733856	Biogenesis of the bc₁ Complex of the Mitochondrial Respiratory Chain Marin-Buera, L, Ott, M, Salvatori, R, Ndi, M, Singh, AP	J Mol Biol	2018
37828827	The functional significance of mitochondrial respiratory chain supercomplexes Ott, M, Barrientos, A, Fontanesi, F, Kohler, A	EMBO Rep	2023
37823874	Structural rather than catalytic role for mitochondrial respiratory chain supercomplexes Cabrera-Orefice, A, Arnold, S, Viscomi, C, Fernandez-Vizarra, E, Brischigliaro, M, Zeviani, M	Elife	2023
28844695	Architecture of Human Mitochondrial Respiratory Megacomplex I₂III₂IV₂ Guo, R, Zong, S, Yang, M, Gu, J, Wu, M	Cell	2017
34969438	Defining the interactome of the human mitochondrial ribosome identifies SMIM4 and TMEM223 as respiratory chain assembly factors Hanitsch, E, Bauermeister, D, Wang, C, Stoldt, S, Poerschke, S, Jakobs, S, Langer, T, Dennerlein, S, Sattmann, J, Richter-Dennerlein, R, Warscheid, B, Rehling, P, Oeljeklaus, S	Elife	2021