DNA Excision Repair Protein ERCC-1 (ERCC1) ELISA Kit for Mouse

ERCC1: a structure-specific DNA repair endonuclease responsible for the 5'-incision during DNA excision repair. Belongs to the ERCC1/RAD10/SWI10 family. Heterodimer composed of ERCC1 and XPF/ERRC4. Defects in ERCC1 are the cause of cerebro-oculo-facio-skeletal syndrome type 4, a degenerative autosomal recessive disorder of prenatal onset affecting the brain, eye and spinal cord. After birth, it leads to brain atrophy, hypoplasia of the corpus callosum, hypotonia, cataracts, microcornea, optic atrophy, progressive joint contractures and growth failure. Facial dysmorphism is a constant feature. Abnormalities of the skull, eyes, limbs, heart and kidney also occur. Low levels of this protein are associated with increased sensitivity to cisplatin. The overall survival of non-small cell lung cancer patients with single-nucleotide polymorphisms at codon 118 receiving platinum-based chemotherapy was significantly improved.

Protein type: DNA repair, damage

Cellular Component: cytoplasm; nuclear chromosome, telomeric region; nucleoplasm; nucleotide-excision repair complex; nucleus; transcription factor TFIID complex

Molecular Function: damaged DNA binding; DNA binding; endonuclease activity; hydrolase activity; nuclease activity; protein binding; protein C-terminus binding; protein domain specific binding; single-stranded DNA binding; single-stranded DNA specific endodeoxyribonuclease activity; structure-specific DNA binding; TATA-binding protein binding

Biological Process: cell development; cell proliferation; chromosome organization and biogenesis; DNA recombination; DNA repair; double-strand break repair; germ cell development; isotype switching; male gonad development; mitotic recombination; multicellular organism growth; multicellular organismal aging; negative regulation of telomere maintenance; nucleotide-excision repair; nucleotide-excision repair, DNA incision, 3'-to lesion; nucleotide-excision repair, DNA incision, 5'-to lesion; oogenesis; post-embryonic hemopoiesis; pyrimidine dimer repair via nucleotide-excision repair; replicative cell aging; response to DNA damage stimulus; response to oxidative stress; response to X-ray; spermatogenesis; syncytium formation; UV protection