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Ubiquinol-cytochrome c reductase complex assembly factor 4 (UQCC4) is a mitochondrial protein required for the assembly and stability of complex III (cytochrome b-c1 complex) in the electron transport chain. Dysfunction impacts mitochondrial oxidative phosphorylation and is associated with diseases such as mucolipidosis III gamma and alpha-2-plasmin inhibitor deficiency. UQCC4 is found in the mitochondria and, while its primary role is in respiratory chain biogenesis, it is reportedly upregulated in some cancers (notably lung cancer), suggesting a potential biomarker function. No approved therapeutics directly target this protein.
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