DNA excision repair protein ERCC-8; Cockayne syndrome WD repeat protein CSA; ERCC8; CKN1; CSA

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

Polyclonal

Rabbit Ig

Rabbit

This ERCC8 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 210-238 amino acids from the Central region of human ERCC8.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-ERCC8 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.

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.

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC)

WB~~1:1000

ERCC8 Antibody (Center) western blot analysis in mouse spleen tissue lysates (35ug/lane).This demonstrates the ERCC8 antibody detected the ERCC8 protein (arrow).

ERCC8 antibody (Center) immunohistochemistry analysis in formalin fixed and paraffin embedded human lung carcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the ERCC8 antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.

44055

excision repair cross-complementation group 8

DNA excision repair protein ERCC-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; DNA helicase activity; protein binding; protein complex binding; ubiquitin-protein ligase activity

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

Briggs, F.B., et al. Am. J. Epidemiol. 172(2):217-224(2010)
Guillem, V.M., et al. Am. J. Hematol. 85(7):482-486(2010)
Monsees, G.M., et al. Breast Cancer Res. Treat. (2010) In press :
Kamenisch, Y., et al. J. Exp. Med. 207(2):379-390(2010)
Laugel, V., et al. Hum. Mutat. 31(2):113-126(2010)

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