Overview

Description

  • NatureRecombinant
  • SourceEscherichia coli
  • Amino Acid Sequence
    • SpeciesHuman

Specifications

Our Abpromise guarantee covers the use of ab83630 in the following tested applications.

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

  • Applications

    ELISA

    Western blot

  • FormLiquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    Preservative: None
    Constituents: 20% Glycerol, 50mM Tris acetate, 1mM EDTA, pH 7.5

General Info

  • Alternative names
    • COFS 4
    • COFS4
    • DNA excision repair protein ERCC 1
    • DNA excision repair protein ERCC-1
    • DNA excision repair protein ERCC1
    • ERCC 1
    • ERCC1
    • ERCC1_HUMAN
    • Excision repair cross complementation group 1
    • Excision repair cross complementing 1
    • Excision Repair Cross Complementing Rodent Repair Deficiency Complementation Group 1
    • Excision repair protein
    • RAD 10
    • RAD10
    • UV 20
    • UV20
    see all
  • FunctionStructure-specific DNA repair endonuclease responsible for the 5'-incision during DNA repair.
  • Involvement in diseaseDefects in ERCC1 are the cause of cerebro-oculo-facio-skeletal syndrome type 4 (COFS4) [MIM:610758]. COFS is a degenerative autosomal recessive disorder of prenatal onset affecting the brain, eye and spinal cord. After birth, it leads to brain atrophy, hypoplasia of the corpus callosum, hypotonia, cataracts, microcornea, optic atrophy, progressive joint contractures and growth failure. Facial dysmorphism is a constant feature. Abnormalities of the skull, eyes, limbs, heart and kidney also occur.
  • Sequence similaritiesBelongs to the ERCC1/RAD10/SWI10 family.
  • Cellular localizationNucleus.
  • Information by UniProt

References for Recombinant Human ERCC1 protein (ab83630)

ab83630 has not yet been referenced specifically in any publications.

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