Overview

Description

  • Nature
    Recombinant
  • Source
    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      MGSSHHHHHH SSGLVPRGSH MGSHMDPGKD KEGVPQPSGP PARKKFVIPL DEDEVPPGVA KPLFRSTQSL PTVDTSAQAA PQTYAEYAIS QPLEGAGATC PTGSEPLAGE TPNQALKPGA KSNSIIVSPR QRGNPVLKFV RNVPWEFGDV IPDYVLGQST CALFLSLRYH NLHPDYIHGR LQSLGKNFAL RVLLVQVDVK DPQQALKELA KMCILADCTL ILAWSPEEAG RYLETYKAYE QKPADLLMEK LEQDFVSRSL EQLIAASRED LALCPGLGPQ KARRLFDVLH EPFLKVP
    • Molecular weight
      33 kDa including tags
    • Amino acids
      1 to 273
    • Tags
      His tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab126690 in the following tested applications.

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

  • Applications

    Mass Spectrometry

    SDS-PAGE

  • Mass spectrometry
    MALDI-TOF
  • Purity
    > 90 % SDS-PAGE.
    purified by using conventional chromatography.
  • Form
    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 8.00
    Constituents: 0.02% DTT, 0.32% Tris HCl, 20% Glycerol, 0.58% Sodium chloride

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
  • Function
    Structure-specific DNA repair endonuclease responsible for the 5'-incision during DNA repair.
  • Involvement in disease
    Defects 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 similarities
    Belongs to the ERCC1/RAD10/SWI10 family.
  • Cellular localization
    Nucleus.
  • Information by UniProt

Images

  • 15% SDS-PAGE using 3µg of ab126690.

References

ab126690 has not yet been referenced specifically in any publications.

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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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