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UQCRQ This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This subunit, together with cytochrome b, binds to ubiquinone. Defects in UQCRQ are a cause of mitochondrial complex III deficiency (MT-C3D). A disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. Clinical features include mitochondrial encephalopathy, psychomotor retardation, ataxia, severe failure to thrive, liver dysfunction, renal tubulopathy, muscle weakness and exercise intolerance. Belongs to the UQCRQ/QCR8 family. Note: This description may include information from UniProtKB.
Protein type: Energy Metabolism - oxidative phosphorylation; Mitochondrial; Oxidoreductase
Chromosomal Location of Human Ortholog: 5q31.1
Cellular Component: mitochondrial inner membrane; mitochondrial respiratory chain complex III; mitochondrion
Molecular Function: ubiquinol-cytochrome-c reductase activity
Biological Process: mitochondrial electron transport, ubiquinol to cytochrome c
Disease: Mitochondrial Complex Iii Deficiency, Nuclear Type 4
Reference #:  O14949 (UniProtKB)
Alt. Names/Synonyms: Complex III subunit 8; Complex III subunit VIII; Cytochrome b-c1 complex subunit 8; low molecular mass ubiquinone-binding protein (9.5kD); QCR8; QP-C; QPC; Ubiquinol-cytochrome c reductase complex 9.5 kDa protein; Ubiquinol-cytochrome c reductase complex ubiquinone-binding protein QP-C; ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5kDa; UQCR7; UQCRQ
Gene Symbols: UQCRQ
Molecular weight: 9,906 Da
Basal Isoelectric point: 10.07  Predict pI for various phosphorylation states
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Protein Structure Not Found.
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