Pathway
Complex III assembly
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).
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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).
What the reference databases state
Statements
21 statements
21 statements · 1 reference database · Subject: Complex III assembly · Sources: Reactome
- BCS1L participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- CYC1 participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- FXN participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- HSCB participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- HSPA9 participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- LETM1 participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- LYRM4 participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- LYRM7 participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- MT-CYB participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- TTC19 participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- UQCC1 participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- UQCC2 participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
Show the remaining 9
- UQCC3 participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- UQCR10 participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- UQCR11 participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- UQCRB participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- UQCRC1 participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- UQCRC2 participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- UQCRFS1 participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- UQCRH participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only
- UQCRQ participates in Complex III assemblyProteinReactome R-HSA-9865881Inferred only