catalog number :
MBS710103
products full name :
Rabbit anti-human ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5kDa polyclonal Antibody
products short name :
ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5kDa
products name syn :
ubiquinol-cytochrome c reductase; complex III subunit VII; 9.5kDa; UQCRQ; QCR8; QP-C; QPC
other names :
Ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5kDa; Cytochrome b-c1 complex subunit 8; cytochrome b-c1 complex subunit 8; complex III subunit 8; complex III subunit VIII; low molecular mass ubiquinone-binding protein (9.5kD); 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; 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
other gene names :
UQCRQ; UQCRQ; QPC; QCR8; QP-C; UQCR7; MC3DN4
uniprot entry name :
QCR8_HUMAN
reactivity :
Human, Mouse, Rat
purity :
Antigen Affinity Purified
tested application :
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
other info1 :
Immunogen: Human UQCRQ
other info2 :
Storage Buffer: PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20 degree C, Avoid freeze / thaw cycles.
ncbi acc num :
AAH01390.1
ncbi mol weight :
9,906 Da
ncbi pathways :
Alzheimer's Disease Pathway (83097); Alzheimer's Disease Pathway (509); Cardiac Muscle Contraction Pathway (93344); Cardiac Muscle Contraction Pathway (93992); Cytochrome Bc1 Complex Pathway (413442); Cytochrome Bc1 Complex Pathway (546500); Electron Transport Chain Pathway (198860); Huntington's Disease Pathway (83100); Huntington's Disease Pathway (512); Metabolic Pathways (132956)
ncbi summary :
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 summary :
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: mitochondrion; mitochondrial inner membrane. Molecular Function: ubiquinol-cytochrome-c reductase activity. Biological Process: cellular metabolic process; hypothalamus development; pons development; hippocampus development; midbrain development; cerebellar Purkinje cell layer development; subthalamus development; pyramidal neuron development; thalamus development. Disease: Mitochondrial Complex Iii Deficiency, Nuclear Type 4