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InnateDB Protein
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IDBP-231628.7
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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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BRCC3
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Protein Name
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BRCA1/BRCA2-containing complex, subunit 3
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Synonyms
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Species
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Homo sapiens
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Ensembl Protein
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ENSP00000381988
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InnateDB Gene
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IDBG-92384 (BRCC3)
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Protein Structure
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| Function |
Metalloprotease that specifically cleaves 'Lys-63'- linked polyubiquitin chains. Does not have activity toward 'Lys- 48'-linked polyubiquitin chains. Component of the BRCA1-A complex, a complex that specifically recognizes 'Lys-63'-linked ubiquitinated histones H2A and H2AX at DNA lesions sites, leading to target the BRCA1-BARD1 heterodimer to sites of DNA damage at double-strand breaks (DSBs). In the BRCA1-A complex, it specifically removes 'Lys-63'-linked ubiquitin on histones H2A and H2AX, antagonizing the RNF8-dependent ubiquitination at double- strand breaks (DSBs). Catalytic subunit of the BRISC complex, a multiprotein complex that specifically cleaves 'Lys-63'-linked ubiquitin in various substrates. Mediates the specific 'Lys-63'- specific deubiquitination associated with the COP9 signalosome complex (CSN), via the interaction of the BRISC complex with the CSN complex. {ECO:0000269PubMed:14636569, ECO:0000269PubMed:16707425, ECO:0000269PubMed:17525341, ECO:0000269PubMed:19202061, ECO:0000269PubMed:19214193, ECO:0000269PubMed:19261746, ECO:0000269PubMed:19261748, ECO:0000269PubMed:19261749}.
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| Subcellular Localization |
Nucleus {ECO:0000269PubMed:18077395, ECO:0000269PubMed:19202061, ECO:0000269PubMed:19261748, ECO:0000269PubMed:19261749}. Note=Localizes at sites of DNA damage at double-strand breaks (DSBs).
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| Disease Associations |
Note=A chromosomal aberration involving BRCC3 is a cause of pro-lymphocytic T-cell leukemia (T-PLL). Translocation t(X;14)(q28;q11) with TCRA.
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| Tissue Specificity |
Heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. Aberrantly expressed in the vast majority of breast tumors. {ECO:0000269PubMed:16707425}.
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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 66 experimentally validated interaction(s) in this database.
They are also associated with 1 interaction(s) predicted by orthology.
| Experimentally validated |
| Total |
66
[view]
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| Protein-Protein |
66
[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 |
IPR000555
JAB1/MPN/MOV34 metalloenzyme domain
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| PFAM |
PF01398
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| PRINTS |
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| PIRSF |
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| SMART |
SM00232
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| TIGRFAMs |
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| Modification |
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| SwissProt |
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| PhosphoSite |
PhosphoSite-P46736
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| TrEMBL |
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| UniProt Splice Variant |
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| Entrez Gene |
79184
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| UniGene |
Hs.735783
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| RefSeq |
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| HUGO |
HGNC:24185
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| OMIM |
300617
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| CCDS |
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| HPRD |
06465
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| IMGT |
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| EMBL |
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| GenPept |
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Version 5.4