Ubiquinol-cytochrome c reductase complex assembly factor 3 (UQCC3)

Ubiquinol-cytochrome c reductase complex assembly factor 3 (UQCC3) is a mitochondrial protein essential for the early assembly and stability of the mitochondrial respiratory chain complex III (cytochrome b-c1 complex), participating in the assembly of mitochondrial respiratory chain supercomplexes. It mediates cytochrome b recruitment and likely stabilization within complex III and is important for ATP production and mitochondrial morphology[2][3][6]. UQCC3 also regulates mitochondrial reactive oxygen species (ROS) generation and participates in cellular adaptation to hypoxia by stabilizing HIF-1α and increasing VEGF expression, thereby promoting angiogenesis, including in both normal development and tumor growth[1]. Clinically, mutations in the UQCC3 gene cause mitochondrial complex III deficiency, leading to severe metabolic disorders. Elevated UQCC3 expression in tumors is associated with poor prognosis, indicating its potential as a biomarker and therapeutic target for cancer, although it is not yet directly targeted by existing drugs[1][2][6].

Other names

C11orf83Assembly factor CBP4 homologUNQ655/PRO1286UNQ655UPF0723 protein C11orf83MC3DN9ubiquinol-cytochrome-c reductase complex assembly factor 3CBP4 ortholog

No drugs described to target UQCC3 directly; mechanism discussed for potential future targeting – inhibition could disrupt angiogenesis via mitochondrial ROS/HIF/VEGF pathway