Anti-CSA / ERCC8 Antibody IHC-plus; ERCC8; CKN1; UVSS2; CSA; Human CSA; ERCC8

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Human ERCC8.

Immunoaffinity Purified

PBS, pH 7, 1% BSA, 20% Glycerol, 0.01% Thimerosal

Aliquot and store at -20 degree C. Minimize freezing and thawing.

Small volumes of anti-CSA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Mutations in CKN1 have been identified in patients with classical Cockayne syndrome, type 1. This syndrome is characterized by cells which are sensitive to ultraviolet radiation and are defective in transcription-coupled repair. The CKN1 product interacts with CSB protein and with p44 protein suggesting CKN1 is involved in transcription. Introducing CKN1 to fibroblasts from Cockayne syndrome patients restored the UV-induced ubiquitination of the large subunit of RNA polymerase II.

Immunohistochemistry (IHC - Paraffin), Immunocytochemistry (ICC), Immunofluorescence (IF), Western Blot (WB)

ICC, IF (1:100 - 1:200), IHC-P (10 ug/ml), WB (1:500 - 1:3000)
Usage: Cross reactivity with mouse has been confirmed in WB only. The suitability of the antibody for use in IHC with mouse samples has not been evaluated at this time. Cross-reactivity with rat and cow has not been experimentally confirmed. The antibody is predicted to cross-react with rat and cow based on sequence homologies of 96% and 95% respectively.

Anti-ERCC8 antibody IHC of human skin. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 10 ug/ml.

Immunofluorescence of paraformaldehyde-fixed A431, using ERCC8(CSA) antibody at 1:200 dilution.

Sample(30 g of whole cell lysate). A:293T. B: A431. 12% SDS PAGE. ERCC8 antibody diluted at 1:500.

Sample (30 ug of whole cell lysate). A: NIH-3T3. 10% SDS PAGE. ERCC8 antibody diluted at 1:1000.

23,182 Da

excision repair cross-complementation group 8

DNA excision repair protein ERCC-8; Cockayne syndrome WD-repeat protein CSA; cockayne syndrome WD repeat protein CSA; excision repair cross-complementing rodent repair deficiency, complementation group 8

DNA excision repair protein ERCC-8

CKN1; CSA??[Similar Products]

ERCC8_HUMAN

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]

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; Helicase; Ubiquitin conjugating system

Chromosomal Location of Human Ortholog: 5q12.1

Cellular Component: nucleoplasm; nucleotide-excision repair complex; nuclear matrix; protein complex; nucleus

Molecular Function: DNA-dependent ATPase activity; protein binding; DNA helicase activity; ubiquitin-protein ligase activity; protein complex binding

Biological Process: proteasomal ubiquitin-dependent protein catabolic process; protein autoubiquitination; positive regulation of DNA repair; protein polyubiquitination; nucleotide-excision repair; transcription-coupled nucleotide-excision repair; response to oxidative stress; DNA repair; response to DNA damage stimulus; response to X-ray; response to UV

Disease: Cockayne Syndrome A; Uv-sensitive Syndrome 2

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