Overview

  • Product name
  • Description
    Rabbit polyclonal to ERCC8
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Mouse, Human
  • Immunogen

    KLH conjugated synthetic peptide between internal sequence amino acids 210-238 of Human ERCC8 (NP_000073.1).

  • Positive control
    • Mouse spleen tissue lysate

Properties

  • Form
    Liquid
  • Storage instructions
    Shipped at 4°C. Store at 4°C (up to 6 months). Store at -20°C long term.
  • Storage buffer
    Preservative: 0.09% Sodium Azide
    Constituents: PBS
  • Concentration information loading...
  • Purity
    Immunogen affinity purified
  • Purification notes
    Purified through a protein A column, followed by peptide affinity purification.
  • Clonality
    Polyclonal
  • Isotype
    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab107290 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/100 - 1/500. Predicted molecular weight: 44 kDa.

Target

  • Function
    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 recruitement 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.
  • Pathway
    Protein modification; protein ubiquitination.
  • Involvement in disease
    Defects in ERCC8 are the cause of Cockayne syndrome type A (CSA) [MIM:216400]. 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.
  • Sequence similarities
    Contains 5 WD repeats.
  • Cellular localization
    Nucleus.
  • Information by UniProt
  • Database links
  • Alternative names
    • CKN1 antibody
    • Cockayne syndrome type A antibody
    • Cockayne syndrome WD repeat protein CSA antibody
    • CSA antibody
    • DNA excision repair protein ERCC-8 antibody
    • DNA excision repair protein ERCC8 antibody
    • ERCC 8 antibody
    • ERCC8 antibody
    • ERCC8_HUMAN antibody
    • excision repair cross-complementing rodent repair deficiency, complementation group 8 antibody
    see all

Images

  • Anti-ERCC8 antibody (ab107290) at 1/100 dilution + Mouse spleen tissue lysate at 35 µg

    Predicted band size : 44 kDa

References

ab107290 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab107290.
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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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