Reactome | Intermediate 0 binds heme and UQCC3

Intermediate 0 binds heme and UQCC3

Stable Identifier

R-HSA-9866061

Type

Reaction [omitted]

Species

Homo sapiens

Compartment

mitochondrial inner membrane

ReviewStatus

5/5

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Heme is incorporated in the Intermediate 0 complex containing nascent cytochrome b (MT-CYB), the exact mechanism being unknown. This first hemylation likely triggers a conformational change that favors the binding of UQCC3 to MT-CYB. The binding of UQCC3 stabilizes the bound heme and allows the subsequent second hemylation (Desmurs et al., 2015; reviewed in Ndi et al., 2018; Zara et al., 2022). This assembly step is inferred from its equivalent in yeast. Defects in UQCC3 lead to an unstable or missing MT-CYTB subunit in Complex I in humans (Wanschers et al., 2014).

Literature References

PubMed ID	Title	Journal	Year
36142449	Assembly of the Multi-Subunit Cytochrome bc₁ Complex in the Yeast Saccharomyces cerevisiae Zara, V, Ferramosca, A, De Blasi, G	Int J Mol Sci	2022
25605331	C11orf83, a mitochondrial cardiolipin-binding protein involved in bc1 complex assembly and supercomplex stabilization Foti, M, Desmurs, M, Lane, L, Raemy, E, Martinou, JC, Bairoch, A, Vaz, FM	Mol Cell Biol	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
25008109	A mutation in the human CBP4 ortholog UQCC3 impairs complex III assembly, activity and cytochrome b stability Szklarczyk, R, Stephan, K, Rodenburg, RJ, Huynen, MA, Rootwelt, T, Helland, IB, Jonckheere, A, Elkamil, A, Suijskens, J, Wanschers, BF, Ott, M, Nijtmans, LG, Smeets, R, van den Brand, MA, Van den Heuvel, L	Hum Mol Genet	2014