Ubiquinol-cytochrome c reductase complex assembly factor 2 (UQCC2)

Ubiquinol-cytochrome c reductase complex assembly factor 2 (UQCC2) is a mitochondrial protein encoded by the UQCC2 gene on chromosome 6. UQCC2 is essential for the proper assembly of complex III (cytochrome b-c1 complex) in the mitochondrial electron transport chain. It functions as an assembly factor that facilitates the expression and stability of the mtDNA-encoded cytochrome b protein, working in concert with UQCC1. UQCC2 localizes to the mitochondrial nucleoid and inner membrane and is critical for cytochrome b biogenesis, affecting mitochondrial ATP production, cellular oxygen consumption, insulin secretion by pancreatic beta-cells, and muscle differentiation. Mutations in UQCC2 lead to complex III deficiency manifesting as neonatal lactic acidosis, intrauterine growth retardation, and renal dysfunction. Currently, UQCC2 is not considered a therapeutic target since no drugs directly interact with it, but it is a key player in mitochondrial biology and monogenic mitochondrial disease.

Other names

C6orf125C6orf126Cbp6M19MC3DN7MNF1bA6B20.2Breast cancer-associated protein SGA-81MMitochondrial nucleoid factor 1Mitochondrial protein M19Cytochrome B protein synthesis 6 homolog (S. cerevisiae)