功能： disease:Defects in UQCRQ are a cause of mitochondrial complex III deficiency (CIII deficiency) [MIM:124000]. Affected individuals display severe psychomotor retardation and extrapyramidal signs. Neurologic features include dystonia, athetoid movements, ataxia, mild axial hypotonia, increased tone, hyperreflexia, and inability to walk unsupported. Serum lactate is mildly elevated and muscle biopsies show a reduction in mitochondrial complex III activity.,function: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.,similarity:Belongs to the UQCRQ/QCR8 family.,subunit:The bc1 complex contains 11 subunits: 3 respiratory subunits (cytochrome b, cytochrome c1 and Rieske/UQCRFS1), 2 core proteins (UQCRC1/QCR1 and UQCRC2/QCR2) and 6 low-molecular weight proteins (UQCRH/QCR6, UQCRB/QCR7, UQCRQ/QCR8, UQCR10/QCR9, UQCR11/QCR10 and a cleavage product of Rieske/UQCRFS1).,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Mitochondrion inner membrane ; Single-pass membrane protein .
功能： disease:Defects in UQCRQ are a cause of mitochondrial complex III deficiency (CIII deficiency) [MIM:124000]. Affected individuals display severe psychomotor retardation and extrapyramidal signs. Neurologic features include dystonia, athetoid movements, ataxia, mild axial hypotonia, increased tone, hyperreflexia, and inability to walk unsupported. Serum lactate is mildly elevated and muscle biopsies show a reduction in mitochondrial complex III activity.,function: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.,similarity:Belongs to the UQCRQ/QCR8 family.,subunit:The bc1 complex contains 11 subunits: 3 respiratory subunits (cytochrome b, cytochrome c1 and Rieske/UQCRFS1), 2 core proteins (UQCRC1/QCR1 and UQCRC2/QCR2) and 6 low-molecular weight proteins (UQCRH/QCR6, UQCRB/QCR7, UQCRQ/QCR8, UQCR10/QCR9, UQCR11/QCR10 and a cleavage product of Rieske/UQCRFS1).,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Mitochondrion inner membrane ; Single-pass membrane protein .
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