NP_082318.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
43,689 Da
NCBI Official Full Name
DNA excision repair protein ERCC-8
NCBI Official Synonym Full Names
excision repaiross-complementing rodent repair deficiency, complementation group 8
NCBI Official Synonym Symbols
Csa; Ckn1; 2410022P04Rik; 2810431L23Rik; 4631412O06Rik; B130065P18Rik [Similar Products]
NCBI Protein Information
DNA excision repair protein ERCC-8; Cockayne syndrome 1 homolog; cockayne syndrome WD repeat protein CSA homolog
UniProt Protein Name
DNA excision repair protein ERCC-8
UniProt Synonym Protein Names
Cockayne syndrome WD repeat protein CSA homolog
UniProt Synonym Gene Names
UniProt Entry Name
ERCC8_MOUSE
UniProt Comments for ERCC8
ERCC8: 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. Defects in ERCC8 are the cause of Cockayne syndrome type A (CSA). Cockayne syndrome is a rare disorder characterized by cutaneous sensitivity to sunlight, abnormal and slow growth, cachectic dwarfism, progeroid appearance, progressive pigmentary retinopathy and sensorineural deafness. There is delayed neural development and severe progressive neurologic degeneration resulting in mental retardation. Two clinical forms are recognized: in the classical form or Cockayne syndrome type 1, the symptoms are progressive and typically become apparent within the first few years or life; the less common Cockayne syndrome type 2 is characterized by more severe symptoms that manifest prenatally. Cockayne syndrome shows some overlap with certain forms of xeroderma pigmentosum. Unlike xeroderma pigmentosum, patients with Cockayne syndrome do not manifest increased freckling and other pigmentation abnormalities in the skin and have no significant increase in skin cancer. Defects in ERCC8 are the cause of UV-sensitive syndrome type 2 (UVSS2). An autosomal recessive disorder characterized by cutaneous photosensitivity and mild freckling in the absence of neurological abnormalities or skin tumors. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: DNA repair, damage; Ubiquitin conjugating system; Helicase
Cellular Component: nucleotide-excision repair complex; protein complex; nuclear matrix; nucleus
Molecular Function: protein complex binding; ubiquitin-protein ligase activity
Biological Process: proteasomal ubiquitin-dependent protein catabolic process; protein autoubiquitination; protein polyubiquitination; positive regulation of DNA repair; nucleotide-excision repair; transcription-coupled nucleotide-excision repair; response to oxidative stress; DNA repair; response to DNA damage stimulus; response to UV; response to X-ray
Research Articles on ERCC8
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Pathways associated with ERCC8 elisa kit
Diseases associated with ERCC8 elisa kit
Organs/Tissues associated with ERCC8 elisa kit
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