Ubiquinol-cytochrome c reductase core protein 2 (UQCRC2)

Ubiquinol-cytochrome c reductase core protein 2 (UQCRC2) is a critical component of mitochondrial Complex III (cytochrome b-c1 complex), a multisubunit enzyme complex of the respiratory chain. The protein is located on the inner mitochondrial membrane, where it stabilizes and is necessary for assembly of Complex III, which catalyzes electron transfer from ubiquinol to cytochrome c. UQCRC2 is part of the peptidase M16 family and is homologous to alpha-mitochondrial processing peptidases, though its established role is structural within the electron transport chain. Pathogenic variants in UQCRC2 cause mitochondrial complex III deficiency, leading to a spectrum of mitochondrial disorders, especially impacting organs with high energetic demand, such as brain, liver, and muscle[1][3][4][6].

Other names

Ubiquinol-cytochrome c reductase complex core protein 2Cytochrome b-c1 complex subunit 2, mitochondrialComplex III subunit 2Core protein IIUQCR2QCR2MC3DN5

N/A (not a current direct therapeutic target; general mechanisms would involve mitochondrial function modulation rather than direct modulation of UQCRC2)