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DNA excision repair protein ERCC1; Excision Repair Cross Complement-1 protein (ERCC1)
Function:
- structure-specific DNA repair endonuclease
- responsible for the 5'-incision during DNA repair
- heterodimer composed of ERCC1 & XPF/ERRC4
- associates with XP A-correcting protein
Structure: belongs to the ERCC1/RAD10/SWI10 family
Compartment: nucleus
Pathology:
- defects in ERCC1 are the cause of cerebro-oculo-facio-skeletal syndrome type 4
Related
DNA repair endonuclease XPF; DNA excision repair protein ERCC-4; DNA repair protein complementing XP-F cells; Xeroderma pigmentosum group F-complementing protein (ERCC4, ERCC11, XPF)
ERCC1 Ag in tissue
General
DNA-binding protein
excision repair cross complement (ERCC) or excision repair cross-complementing rodent repair deficiency, complementation group
Properties
SIZE: MW = 33 kD
entity length = 297 aa
COMPARTMENT: cell nucleus
MOTIF: nuclear translocation signal {17-23}
binding site
SITE: 93-120
FOR-BINDING-OF: DNA repair protein complementing XP-A cells
DNA-binding motif
SITE: 134-156
binding site
SITE: 224-297
FOR-BINDING-OF: DNA repair endonuclease XPF
Database Correlations
OMIM correlations
UniProt P07992
PFAM correlations
Entrez Gene 2067
Kegg hsa:2067
References
- UniProt :accession P07992
- Li et al PNAS 91:5012 1994
- Entrez Gene :accession 2067
- Atlas of Genetics & Cytogenetics in Oncology & Haematology
http://atlasgeneticsoncology.org/genes/LYL1ID51ch19p13.html
- NIEHS-SNPs
http://egp.gs.washington.edu/data/ercc1/
Component-of
ERCC1/XPF heterodimer