NP_055217.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
cytochrome b-c1 complex subunit 8
NCBI Official Synonym Full Names
ubiquinol-cytochrome c reductase complex III subunit VII
NCBI Protein Information
cytochrome b-c1 complex subunit 8
UniProt Protein Name
Cytochrome b-c1 complex subunit 8
UniProt Synonym Protein Names
Complex III subunit 8; Complex III subunit VIII; Ubiquinol-cytochrome c reductase complex 9.5 kDa protein; Ubiquinol-cytochrome c reductase complex ubiquinone-binding protein QP-C
UniProt Entry Name
QCR8_HUMAN
NCBI Summary for UQCRQ
This gene encodes a ubiquinone-binding protein of low molecular mass. This protein is a small core-associated protein and a subunit of ubiquinol-cytochrome c reductase complex III, which is part of the mitochondrial respiratory chain. [provided by RefSeq, Jul 2008]
UniProt Comments for UQCRQ
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.
Protein type: Oxidoreductase; Mitochondrial; Energy Metabolism - oxidative phosphorylation
Chromosomal Location of Human Ortholog: 5q31.1
Cellular Component: mitochondrial inner membrane; mitochondrion
Molecular Function: ubiquinol-cytochrome-c reductase activity
Biological Process: cellular metabolic process; cerebellar Purkinje cell layer development; hippocampus development; hypothalamus development; midbrain development; pons development; pyramidal neuron development; subthalamus development; thalamus development
Disease: Mitochondrial Complex Iii Deficiency, Nuclear Type 4
Research Articles on UQCRQ
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Products associated with anti-UQCRQ antibody
Pathways associated with anti-UQCRQ antibody
Diseases associated with anti-UQCRQ antibody
Organs/Tissues associated with anti-UQCRQ antibody
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