ERCC8

Entrez Summary: This gene encodes a WD repeat protein, which interacts with Cockayne syndrome type B (CSB) protein and with p44 protein, a subunit of the RNA polymerase II transcription factor IIH. Mutations in this gene have been identified in patients with hereditary disease Cockayne syndrome (CS). CS cells are abnormally sensitive to ultraviolet radiation and are defective in the repair of transcriptionally active genes. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2014].
UniProt Summary: Substrate-recognition component of the CSA complex, a DCX (DDB1-CUL4-X-box) E3 ubiquitin-protein ligase complex, involved in transcription-coupled nucleotide excision repair. The CSA complex (DCX(ERCC8) complex) promotes the ubiquitination and subsequent proteasomal degradation of ERCC6 in a UV-dependent manner; ERCC6 degradation is essential for the recovery of RNA synthesis after transcription-coupled repair. It is required for the recruitment of XAB2, HMGN1 and TCEA1/TFIIS to a transcription- coupled repair complex which removes RNA polymerase II-blocking lesions from the transcribed strand of active genes. {ECO:0000269|PubMed:16751180, ECO:0000269|PubMed:16916636, ECO:0000269|PubMed:16964240}.

Pfam Domains GO Terms

Compound Hit Most Correlated Genes in Chemogenomics Tissues where Essential in the Avana Dataset (DepMap 20Q1)

Screen	Score
4-Nitroquinoline-1-oxide 0.5μM R08 exp446	-3.23
Nutlin-3A 1.6μM R06 exp294	-2.16
Diepoxybutane 3μM R07 exp354	-2.14
Nocodazole 0.2μM R02 exp64	-2.1
Menadione 5μM R06 exp292	-2.03
Tunicamycin 0.04 to 0.075μM on day4 R07 exp419	-1.94

Global Fraction of Cell Lines Where Essential: 0/739

Tissue	Fraction Of Cell Lines Where Essential
1290807.0	0/1
909776.0	0/1
bile duct	0/28
blood	0/28
bone	0/26
breast	0/33
central nervous system	0/56
cervix	0/4
colorectal	0/17
esophagus	0/13
fibroblast	0/1
gastric	0/16
kidney	0/21
liver	0/20
lung	0/75
lymphocyte	0/16
ovary	0/26
pancreas	0/24
peripheral nervous system	0/16
plasma cell	0/15
prostate	0/1
skin	0/24
soft tissue	0/9
thyroid	0/2
upper aerodigestive	0/22
urinary tract	0/29
uterus	0/5

Expression Distribution

nucleotide-excision repair complex
Cul4A-RING E3 ubiquitin ligase complex
response to auditory stimulus
Cul4-RING E3 ubiquitin ligase complex
response to X-ray
DNA-dependent ATPase activity
DNA helicase activity
protein autoubiquitination
positive regulation of DNA repair
transcription-coupled nucleotide-excision repair
positive regulation of response to DNA damage stimulus
nuclear matrix
nucleotide-excision repair
DNA duplex unwinding
DNA geometric change
regulation of DNA repair
perikaryon
response to UV
response to ionizing radiation
positive regulation of DNA metabolic process
response to mechanical stimulus
regulation of response to DNA damage stimulus
ubiquitin-protein transferase activity
protein-containing complex binding
DNA conformation change
protein polyubiquitination
response to light stimulus
proteasome-mediated ubiquitin-dependent protein catabolic process
proteasomal protein catabolic process
regulation of DNA metabolic process
post-translational protein modification
response to oxidative stress
response to radiation
DNA repair
ubiquitin-dependent protein catabolic process
modification-dependent protein catabolic process
modification-dependent macromolecule catabolic process
proteolysis involved in cellular protein catabolic process
protein-containing complex
cellular protein catabolic process