Western Blot (WB)
(WB Suggested Anti-ERCC8 Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:12500Positive Control: Hela cell lysate)
Rabbit ERCC8 Polyclonal Antibody | anti-ERCC8 antibody
ERCC8 antibody - middle region
Western Blot (WB)
(WB Suggested Anti-ERCC8 Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:12500Positive Control: Hela cell lysate)
Western Blot (WB)
(WB Suggested Anti-ERCC8 Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:12500Positive Control: Hela cell lysate)
Target Description: ERCC8 is 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).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.
NCBI and Uniprot Product Information
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
Research Articles on ERCC8
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Product Notes
The ERCC8 ercc8 (Catalog #AAA3213658) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The ERCC8 antibody - middle region reacts with Cow, Dog, Guinea Pig, Horse, Human, Mouse, Pig, Rabbit, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's ERCC8 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Researchers should empirically determine the suitability of the ERCC8 ercc8 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. The amino acid sequence is listed below: Synthetic peptide located within the following region: VRRASGCLIT LDQHNGKKSQ AVESANTAHN GKVNGLCFTS DGLHLLTVGT. It is sometimes possible for the material contained within the vial of "ERCC8, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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