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ERCC4 Structure-specific DNA repair endonuclease responsible for the 5-prime incision during DNA repair. Involved in homologous recombination that assists in removing interstrand cross-link. Defects in ERCC4 are the cause of xeroderma pigmentosum complementation group F (XP-F); also known as xeroderma pigmentosum VI (XP6). XP-F is an autosomal recessive disease characterized by hypersensitivity of the skin to sunlight followed by high incidence of skin cancer and frequent neurologic abnormalities. Defects in ERCC4 are a cause of XFE progeroid syndrome (XFEPS). This syndrome is illustrated by one patient who presented with dwarfism, cachexia and microcephaly. Belongs to the XPF family. Note: This description may include information from UniProtKB.
Protein type: DNA repair, damage; Deoxyribonuclease; EC 3.1.-.-
Chromosomal Location of Human Ortholog: 16p13.12
Cellular Component: chromosome, telomeric region; nuclear chromosome, telomeric region; nucleoplasm; nucleotide-excision repair complex; nucleus
Molecular Function: damaged DNA binding; DNA binding; endodeoxyribonuclease activity; protein binding; protein C-terminus binding; protein N-terminus binding; single-stranded DNA binding
Biological Process: DNA repair; double-strand break repair via homologous recombination; double-strand break repair via nonhomologous end joining; negative regulation of telomerase activity; 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; resolution of meiotic joint molecules as recombinants; response to UV; telomere maintenance; transcription-coupled nucleotide-excision repair
Disease: Fanconi Anemia, Complementation Group Q; Tracheoesophageal Fistula With Or Without Esophageal Atresia; Xeroderma Pigmentosum, Complementation Group F; Xfe Progeroid Syndrome
Reference #:  Q92889 (UniProtKB)
Alt. Names/Synonyms: DNA excision repair protein ERCC-4; DNA repair endonuclease XPF; DNA repair protein complementing XP-F cells; ERCC11; ERCC4; excision repair cross-complementing rodent repair deficiency, complementation group 4; excision-repair, complementing defective, in Chinese hamster; RAD1; Xeroderma pigmentosum group F-complementing protein; xeroderma pigmentosum, complementation group F; XPF
Gene Symbols: ERCC4
Molecular weight: 104,486 Da
Basal Isoelectric point: 6.5  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
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