InnateDB Protein
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IDBP-29386.6
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Last Modified
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2014-10-13 [Report errors or provide feedback]
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Gene Symbol
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UQCRB
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Protein Name
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ubiquinol-cytochrome c reductase binding protein
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Synonyms
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MC3DN3; QCR7; QP-C; QPC; UQBC; UQBP; UQCR6; UQPC;
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Species
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Homo sapiens
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Ensembl Protein
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ENSP00000287022
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InnateDB Gene
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IDBG-29384 (UQCRB)
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Protein Structure
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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 component is involved in redox-linked proton pumping.
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Subcellular Localization |
Mitochondrion inner membrane.
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Disease Associations |
Mitochondrial complex III deficiency, nuclear 3 (MC3DN3) [MIM:615158]: 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. {ECO:0000269PubMed:12709789}. Note=The disease is caused by mutations affecting the gene represented in this entry.
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Tissue Specificity |
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Comments |
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Number of Interactions
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This gene and/or its encoded proteins are associated with 26 experimentally validated interaction(s) in this database.
They are also associated with 1 interaction(s) predicted by orthology.
Experimentally validated |
Total |
26
[view]
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Protein-Protein |
26
[view]
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Protein-DNA |
0
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Protein-RNA |
0
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DNA-DNA |
0
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RNA-RNA |
0
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DNA-RNA |
0
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Predicted by orthology |
Total |
1 [view]
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Molecular Function |
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Biological Process |
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Cellular Component |
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PDB ID |
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InterPro |
IPR003197
Cytochrome b-c1 complex subunit 7
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PFAM |
PF02271
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PRINTS |
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PIRSF |
PIRSF000022
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SMART |
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TIGRFAMs |
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Modification |
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SwissProt |
P14927
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PhosphoSite |
PhosphoSite-P14927
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TrEMBL |
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UniProt Splice Variant |
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Entrez Gene |
7381
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UniGene |
Hs.663220
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RefSeq |
NP_006285
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HUGO |
HGNC:12582
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OMIM |
191330
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CCDS |
CCDS6269
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HPRD |
11770
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IMGT |
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EMBL |
AP003465
BC005230
BU535281
CH471060
CR542176
CR542196
M22348
M26700
M26701
M26706
M26707
M26730
M35761
M37387
X13585
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GenPept |
AAA60235
AAA60236
AAA60237
AAA60238
AAA60361
AAH05230
CAA31926
CAG46973
CAG46993
EAW91749
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