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Protein Page:
UQCRQ (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
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: Mitochondrial; Oxidoreductase; Energy Metabolism - oxidative phosphorylation
Cellular Component: mitochondrion; mitochondrial inner membrane
Molecular Function: ubiquinol-cytochrome-c reductase activity
Biological Process: cellular metabolic process; hypothalamus development; pons development; midbrain development; hippocampus development; cerebellar Purkinje cell layer development; subthalamus development; pyramidal neuron development; thalamus development
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
Select Structure to View Below

UQCRQ

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 S16-p TRMRHVIsySLSPFE
0 1 Y17-p RMRHVIsySLSPFEQ
0 1 T32-p RAYPHVFtkGIPNVL
0 48 K33 AYPHVFtKGIPNVLR
0 3 K33-ub AYPHVFtkGIPNVLR
0 2 K33 AYPHVFtKGIPNVLR
0 16 K82-ac PAAYENDk_______
  mouse

 
S16 ARIRHVISYSLSPFE
Y17 RIRHVISYSLSPFEQ
S32 RAFPSYFSkGIPNVL
K33-ac AFPSYFSkGIPNVLR
K33-ub AFPSYFSkGIPNVLR
K33-sc AFPSYFSkGIPNVLR
K82-ac PAMYENDk_______
  cow

 
T16 TRVRHVITYSLSPFE
Y17 RVRHVITYSLSPFEQ
S32 RAFPHYFSKGIPNVL
K33 AFPHYFSKGIPNVLR
K33 AFPHYFSKGIPNVLR
K33 AFPHYFSKGIPNVLR
R82 PAAYENDR_______
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