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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. Note: This description may include information from UniProtKB.
Protein type: DNA repair, damage
Chromosomal Location of Human Ortholog: 19q13.32
Cellular Component: cytosol; nuclear chromosome, telomeric region; nucleoplasm; nucleotide-excision repair complex
Molecular Function: damaged DNA binding; DNA binding; protein binding; protein C-terminus binding; protein domain specific binding; single-stranded DNA binding; single-stranded DNA specific endodeoxyribonuclease activity
Biological Process: DNA recombination; DNA repair; double-strand break repair via nonhomologous end joining; meiotic mismatch repair; mitotic recombination; negative regulation of telomere maintenance; nucleotide-excision repair; nucleotide-excision repair, DNA incision; nucleotide-excision repair, DNA incision, 3'-to lesion; nucleotide-excision repair, DNA incision, 5'-to lesion; nucleotide-excision repair, preincision complex stabilization; response to oxidative stress; transcription-coupled nucleotide-excision repair
Disease: Cerebrooculofacioskeletal Syndrome 4
Reference #:  P07992 (UniProtKB)
Alt. Names/Synonyms: COFS4; DNA excision repair protein ERCC-1; ERCC1; excision repair cross-complementing rodent repair deficiency, complementation group 1 (includes overlapping antisense sequence); UV20
Gene Symbols: ERCC1
Molecular weight: 32,562 Da
Basal Isoelectric point: 5.9  Predict pI for various phosphorylation states
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