UQCC3

UQCC3 (Ubiquinol-Cytochrome C Reductase Complex Assembly Factor 3) is a protein that in humans is encoded by the UQCC3 gene. The protein encoded by this gene is part of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is a respiratory chain that generates an electron transport gradient for ATP synthesis.

Function

The UQCC3 protein is an assembly factor for the mitochondrial respiratory chain complex III. It is involved in the assembly of the cytochrome b-c1 complex, which is part of the mitochondrial respiratory chain that generates an electron transport gradient used for ATP synthesis.

Clinical significance

Mutations in the UQCC3 gene have been associated with mitochondrial diseases, including mitochondrial complex III deficiency, a disorder that affects many parts of the body and can cause a variety of health problems.

See also

• Mitochondrial complex III
• Mitochondrial disease
• Electron transport chain

References

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This article incorporates text from the United States National Library of Medicine, which is in the public domain.