Rabbit anti-Human, mouse ERCC8 Polyclonal Antibody | anti-ERCC8 antibody

ERCC8 Antibody (Center)

Cat. Num. AAA9207552

• Gene Names: ERCC8; CSA; CKN1; UVSS2
• Reactivity: Human, mouse
• Applications: Western Blot, ELISA, Immunohistochemistry
• Purity: Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
• Synonyms: ERCC8; Polyclonal Antibody; ERCC8 Antibody (Center); DNA excision repair protein ERCC-8; Cockayne syndrome WD repeat protein CSA; CKN1; CSA; anti-ERCC8 antibody

• Host: Rabbit
• Reactivity: Human, mouse
• Clonality: Polyclonal
• Isotype: Rabbit Ig
• Specificity: 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.
• Purity/Purification: Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
• Form/Format: 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.
• Concentration: Vial Concentration: 0.5 (varies by lot)
• Sequence Positions: 210-238
• Sequence Length: 205

• Applicable Applications for anti-ERCC8 antibody: Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC)
• Application Notes: WB~~1:1000
• Antigen Type: Synthetic Peptide
• Antigen Source: HUMAN
• Preparation and Storage: 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.

Western Blot (WB)

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

Immunohistochemistry (IHC)

(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.)

Related Product Information for anti-ERCC8 antibody

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.

References

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)

NCBI and Uniprot Product Information

• NCBI GI #: 4557467
• NCBI GeneID: 1161
• NCBI Accession #: NP_000073.1
• NCBI GenBank Nucleotide #: NM_000082.3
• UniProt Accession #: Q13216; Q6FHX5; Q96GB9; B2RB64
• Molecular Weight: 44055
• NCBI Official Full Name: DNA excision repair protein ERCC-8 isoform 1
• NCBI Official Synonym Full Names: excision repair cross-complementation group 8
• NCBI Official Symbol: ERCC8
• NCBI Official Synonym Symbols: CSA; CKN1; UVSS2
• NCBI Protein Information: DNA excision repair protein ERCC-8
• UniProt Protein Name: DNA excision repair protein ERCC-8
• Protein Family: DNA excision repair protein
• UniProt Gene Name: ERCC8
• UniProt Synonym Gene Names: CKN1; CSA
• UniProt Entry Name: ERCC8_HUMAN

NCBI Description

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]

Uniprot Description

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