| Primary Identifier | IPR060204 | Type | Domain |
| Short Name | FANCG_N |
| description | This entry represents the N-terminal TPR domain of Fanconi anemia group G protein (FANCG). FANCG contains four tetratricopeptide repeat (TPR) domains that mediate protein-protein interactions. The specific function of this N-terminal TPR domain remains to be determined.Fanconi anemia (FA) is a human disorder characterised by cancer susceptibility and cellular sensitivity to DNA crosslinks and other damages. This entry represents FANCG, which is part of the FA core complex required for the monoubiquitylation of FANCD2 and the FANCI heterodimer. FANCG is a DNA repair protein that operates in interstrand DNA cross-link repair and maintenance of normal chromosome stability [, ]. The protein belongs to the multisubunit FA complex composed of FANCA, FANCB, FANCC, FANCE, FANCF, FANCG, FANCL and FANCM. The protein localises to the nucleus and is highly expressed in testis and thymus. Mutations in FANCG cause Fanconi anemia complementation group G. The FA complex coordinates activities of the downstream DNA repair pathway including nucleotide excision repair, translesion synthesis, and homologous recombination []. Besides being part of the FA complex, FANCG also promotes formation of a protein complex containing BRCA2, FANCD2 and XRCC3 []. |
