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Epigenetics and Nuclear Signaling DNA / RNA DNA Damage & Repair Nucl. Excision Repair
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Recombinant Human CSB protein (ab114506)

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SDS-PAGE - Recombinant Human CSB protein (ab114506)

    Key features and details

    • Expression system: Wheat germ
    • Suitable for: ELISA, SDS-PAGE, WB

    Description

    • Product name

      Recombinant Human CSB protein
    • Expression system

      Wheat germ
    • Accession

      Q03468
    • Protein length

      Protein fragment
    • Animal free

      No
    • Nature

      Recombinant
      • Species

        Human
      • Sequence

        RARNHLILPERLESESGHLQEASALLPTTEHDDLLVEMRNFIAFQAHTDG QASTREILQEFESKLSASQSCVFRELLRNLCTFHRTSGGEGIWKLKPEYC
      • Predicted molecular weight

        37 kDa including tags
      • Amino acids

        1394 to 1493

    Specifications

    Our Abpromise guarantee covers the use of ab114506 in the following tested applications.

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

    • Applications

      ELISA

      SDS-PAGE

      Western blot

    • Form

      Liquid
    • Concentration information loading...

    Preparation and Storage

    • Stability and Storage

      Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

      pH: 8.00
      Constituents: 0.3% Glutathione, 0.79% Tris HCl

    General Info

    • Alternative names

      • 4732403I04
      • ARMD 5
      • ARMD5
      • ATP dependent helicase ERCC6
      • ATP-dependent helicase ERCC6
      • C130058G22Rik
      • CKN 2
      • CKN2
      • Cockayne syndrome B protein
      • Cockayne syndrome group B protein
      • Cockayne syndrome protein CSB
      • COFS
      • COFS1
      • CS group B correcting
      • CSB
      • DNA excision repair protein ERCC 6
      • DNA excision repair protein ERCC-6
      • ERCC 6
      • ERCC excision repair 6 chromatin remodeling factor
      • ERCC6
      • ERCC6_HUMAN
      • Excision repair cross complementing rodent repair deficiency, complementation group 6
      • OTTHUMP00000019581
      • RAD26
      • Rad26 homolog
      • UVSS1
      see all
    • Function

      Essential factor involved in transcription-coupled nucleotide excision repair which allows RNA polymerase II-blocking lesions to be rapidly removed from the transcribed strand of active genes. Upon DNA-binding, it locally modifies DNA conformation by wrapping the DNA around itself, thereby modifying the interface between stalled RNA polymerase II and DNA. It is required for transcription-coupled repair complex formation. It recruits the CSA complex (DCX(ERCC8) complex), nucleotide excision repair proteins and EP300 to the at sites of RNA polymerase II-blocking lesions.
    • Involvement in disease

      Defects in ERCC6 are the cause of Cockayne syndrome type B (CSB) [MIM:133540]. 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 ERCC6 are the cause of cerebro-oculo-facio-skeletal syndrome type 1 (COFS1) [MIM:214150]; also known as COFS syndrome or Pena-Shokeir syndrome type 2. 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.
      Defects in ERCC6 are a cause of De Sanctis-Cacchione syndrome (DSC) [MIM:278800]; also known as xerodermic idiocy. DSC is an autosomal recessive syndrome consisting of xeroderma pigmentosum associated with mental retardation, retarded growth, gonadal hypoplasia and sometimes neurologic complications.
      Note=A genetic variation in the 5-prime flanking region of ERCC6 has been shown to be associated with susceptibility to age-related macular degeneration.
      Defects in ERCC6 are a cause of UV-sensitive syndrome (UVS) [MIM:600630]. UVS is a rare autosomal recessive disorder characterized by photosensitivity and mild freckling but without neurological abnormalities or skin tumors.
    • Sequence similarities

      Belongs to the SNF2/RAD54 helicase family.
      Contains 1 helicase ATP-binding domain.
      Contains 1 helicase C-terminal domain.
    • Domain

      A C-terminal ubiquitin-binding domain (UBD) is essential for transcription-coupled nucleotide excision repair to proceed.
    • Post-translational
      modifications

      Phosphorylated upon DNA damage, probably by ATM or ATR.
      Ubiquitinated at the C-terminus. Ubiquitination by the CSA complex leads to ERCC6 proteasomal degradation in a UV-dependent manner.
    • Cellular localization

      Nucleus.
    • Target information above from: UniProt accession Q03468 The UniProt Consortium
      The Universal Protein Resource (UniProt) in 2010
      Nucleic Acids Res. 38:D142-D148 (2010) .

      Information by UniProt

    Images

    • SDS-PAGE - Recombinant Human CSB protein (ab114506)
      SDS-PAGE - Recombinant Human CSB protein (ab114506)
      ab114506 analysed by 12.5% SDS-PAGE and stained with Coomassie Blue.

    Protocols

    To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.

    Click here to view the general protocols

    Datasheets and documents

    • SDS download

    • Datasheet download

      Download

    References (0)

    Publishing research using ab114506? Please let us know so that we can cite the reference in this datasheet.

    ab114506 has not yet been referenced specifically in any publications.

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