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

ERCC8 (DNA Excision Repair Protein ERCC-8, Cockayne Syndrome WD Repeat Protein CSA, CKN1, CSA) (AP)

Cat. Num. AAA6377635

• Gene Names: ERCC8; CSA; CKN1; UVSS2
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: ERCC8; Polyclonal Antibody; ERCC8 (DNA Excision Repair Protein ERCC-8; Cockayne Syndrome WD Repeat Protein CSA; CKN1; CSA) (AP); anti-ERCC8 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human ERCC8.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phosphatase (AP).

• Applicable Applications for anti-ERCC8 antibody: Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Full length human ERCC8, aa1-396 (NP_000073.1).
• Immunogen Sequence: MLGFLSARQTGLEDPLRLRRAESTRRVLGLELNKDRDVERIHGGGINTLDIEPVEGRYMLSGGSDGVIVLYDLENSSRQSYYTCKAVCSIGRDHPDVHRYSVETVQWYPHDTGMFTSSSFDKTLKVWDTNTLQTADVFNFEETVYSHHMSPVSTKHCLVAVGTRGPKVQLCDLKSGSCSHILQGHRQEILAVSWSPRYDYILATASADSRVKLWDVRRASGCLITLDQHNGKKSQAVESANTAHNGKVNGLCFTSDGLHLLTVGTDNRMRLWNSSNGENTLVNYGKVCNNSKKGLKFTVSCGCSSEFVFVPYGSTIAVYTVYSGEQITMLKGHYKTVDCCVFQSNFQELYSGSRDCNILAWVPSLYEPVPDDDETTTKSQLNPAFEDAWSSSDEEG
• Conjugate: AP
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western Blot analysis of ERCC8 expression in transfected 293T cell line by ERCC8 polyclonal antibody. Lane 1: ERCC8 transfected lysate (44.1kD). Lane 2: Non-transfected lysate.)

Product Categories/Family for anti-ERCC8 antibody

Antibodies; Transcription Factors

NCBI and Uniprot Product Information

• NCBI GI #: 4557467
• NCBI GeneID: 1161
• NCBI Accession #: NP_000073.1
• NCBI GenBank Nucleotide #: NM_000082.2
• UniProt Accession #: Q13216; Q6FHX5; Q96GB9; B2RB64
• Molecular Weight: 23,182 Da
• 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; Cockayne syndrome WD-repeat protein CSA; cockayne syndrome WD repeat protein CSA; excision repair cross-complementing rodent repair deficiency, complementation group 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; 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