Cytochrome b-c1 complex subunit 8 (UQCRQ)

Cytochrome b-c1 complex subunit 8 (UQCRQ) is a small, low-molecular-mass protein that forms part of the ubiquinol-cytochrome c reductase complex (Complex III) in the mitochondrial respiratory chain[1][3][7]. UQCRQ acts as a ubiquinone-binding protein and is integral for the electron transfer from ubiquinol to cytochrome c, a process that drives proton translocation across the mitochondrial inner membrane and is essential for oxidative phosphorylation and ATP generation. Mutations in UQCRQ are linked to mitochondrial complex III deficiency, manifesting in various metabolic and neurometabolic diseases[1][3]. Although there are no drugs targeting UQCRQ specifically, inhibitors of Complex III can impact this protein’s function and mitochondrial bioenergetics[7].

Other names

Cytochrome b-c1 complex subunit 8UQCRQQCR8QP-CQCR7Complex III subunit 8Complex III subunit VIIUbiquinol-cytochrome c reductase complex 9.5 kDa proteinUbiquinol-cytochrome c reductase complex ubiquinone-binding protein QP-C9.5 kDa proteinlow molecular mass ubiquinone-binding protein (9.5kD)

Inhibition of Complex III disrupts electron flow from ubiquinol to cytochrome c, collapsing the mitochondrial membrane potential and reducing ATP synthesis