UQCR11

UQCR11 (Ubiquinol-Cytochrome C Reductase, Complex III Subunit XI) is a protein that in humans is encoded by the UQCR11 gene. This protein is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome bc1 complex), which is part of the mitochondrial respiratory chain.

Function

The UQCR11 protein is a subunit of the ubiquinol-cytochrome c reductase complex, which consists of the products of one mitochondrially encoded gene (cytochrome b) and ten nuclear genes. This complex is located in the mitochondrial inner membrane and is involved in the transfer of electrons from ubiquinol to cytochrome c, a critical step in the mitochondrial ATP synthesis pathway.

Clinical significance

Mutations in the UQCR11 gene have been associated with mitochondrial complex III deficiency, a disorder that can cause a variety of clinical manifestations, including growth retardation, hepatomegaly, hypoglycemia, lactic acidosis, hypotonia, and encephalopathy. Patients with this disorder may also have a decreased amount of the ubiquinol-cytochrome c reductase complex in their mitochondria.

See also

• Mitochondrial disease
• Cytochrome c
• Ubiquinol

References

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