Rabbit anti-Human ERCC5 Polyclonal Antibody

Uniprot Id

P28715

Target Species

Human

Target Name

ERCC5

Target Full Name

DNA excision repair protein ERCC-5

Target Function

Single-stranded structure-specific DNA endonuclease involved in DNA excision repair. Makes the 3'incision in DNA nucleotide excision repair (NER). Binds and bends DNA repair bubble substrate and breaks base stacking at the single-strand/double-strand DNA junction of the DNA bubble. Plays a role in base excision repair (BER) by promoting the binding of DNA glycosylase NTHL1 to its substrate and increasing NTHL1 catalytic activity that removes oxidized pyrimidines from DNA. Involved in transcription-coupled nucleotide excision repair (TCR) which allows RNA polymerase II-blocking lesions to be rapidly removed from the transcribed strand of active genes. Functions during the initial step of TCR in cooperation with ERCC6/CSB to recognized stalled RNA polymerase II. Also, stimulates ERCC6/CSB binding to the DNA repair bubble and ERCC6/CSB ATPase activity. Required for DNA replication fork maintenance and preservation of genomic stability. Involved in homologous recombination repair (HRR) induced by DNA replication stress by recruiting RAD51, BRCA2, and PALB2 to the damaged DNA site. During HRR, binds to the replication fork with high specificity and stabilizes it. Also, acts upstream of HRR, to promote the release of BRCA1 from DNA.

Target Involvement

Xeroderma pigmentosum complementation group G (XP-G); Cerebro-oculo-facio-skeletal syndrome 3 (COFS3)

Target Subcellular Location

Nucleus. Chromosome.

Target Protein Families

XPG/RAD2 endonuclease family, XPG subfamily

Target Synonyms

COFS 3; COFS3; DNA excision repair protein ERCC 5; DNA excision repair protein ERCC-5; DNA excision repair protein ERCC5; DNA repair protein complementing XP G cells; DNA repair protein complementing XP-G cells; DNA repair protein complementing XPG cells; ERCC 5; ERCC5; ERCC5_HUMAN; ERCM 2; ERCM2; Excision repair cross complementation group 5; Excision Repair Cross Complementing Rodent Repair Deficiency; Excision repair cross complementing rodent repair deficiency complementation group 5; Excision repair protein; OTTHUMP00000064902; UVDR; Xeroderma Pigmentosum Complementation Group G; Xeroderma pigmentosum complementation group G protein; Xeroderma pigmentosum group G complementing protein; Xeroderma pigmentosum group G-complementing protein; XPG; XPG complementing protein; XPGC

Target Background

This gene encodes a single-strand specific DNA endonuclease that makes the 3' incision in DNA excision repair following UV-induced damage. The protein may also function in other cellular processes, including RNA polymerase II transcription, and transcription-coupled DNA repair. Mutations in this gene cause xeroderma pigmentosum complementation group G (XP-G), which is also referred to as xeroderma pigmentosum VII (XP7), a skin disorder characterized by hypersensitivity to UV light and increased susceptibility for skin cancer development following UV exposure. Some patients also develop Cockayne syndrome, which is characterized by severe growth defects, cognitive disability, and cachexia. Read-through transcription exists between this gene and the neighboring upstream BIVM (basic, immunoglobulin-like variable motif containing) gene.