Overview

Description

  • NatureRecombinant
  • SourceWheat germ
  • Amino Acid Sequence
    • AccessionP19447
    • SpeciesHuman
    • SequenceAPGNDPQEAVPSAAGKQVDESGTKVDEYGAKDYRLQMPLKDDHTSRPLWV APDGHIFLEAFSPVYKYAQDFLVAIAEPVCRPTHVHEYKLTAYSLYAAVS
    • Molecular weight37 kDa including tags
    • Amino acids29 to 128

Specifications

Our Abpromise guarantee covers the use of ab114795 in the following tested applications.

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

  • Applications

    Western blot

    SDS-PAGE

    ELISA

  • FormLiquid
  • Additional notesProtein concentration is above or equal to 0.05 mg/ml.
    This protein is best used within three months from the date of receipt.
  • 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
    • Basic transcription factor 2 89 kDa subunit
    • BTF 2
    • BTF2
    • BTF2 p89
    • DNA excision repair protein ERCC-3
    • DNA repair protein complementing XP-B cells
    • ERCC 3
    • ercc3
    • ERCC3_HUMAN
    • Excision Repair Cross-complementing Rodent Repair deficiency complementation Group 3
    • GTF2H
    • RAD 25
    • RAD25
    • TFIIH
    • TFIIH 89 kDa subunit
    • TFIIH basal transcription factor complex 89 kDa subunit
    • TFIIH basal transcription factor complex helicase XPB subunit
    • TFIIH p89
    • Xeroderma pigmentosum group B-complementing protein
    see all
  • FunctionATP-dependent 3'-5' DNA helicase, component of the core-TFIIH basal transcription factor, involved in nucleotide excision repair (NER) of DNA and, when complexed to CAK, in RNA transcription by RNA polymerase II. Acts by opening DNA either around the RNA transcription start site or the DNA damage.
  • Involvement in diseaseDefects in ERCC3 are the cause of xeroderma pigmentosum complementation group B (XP-B) [MIM:610651]; also known as xeroderma pigmentosum II (XP2) or XP group B (XPB) or xeroderma pigmentosum group B combined with Cockayne syndrome (XP-B/CS). Xeroderma pigmentosum is an autosomal recessive pigmentary skin disorder characterized by solar hypersensitivity of the skin, high predisposition for developing cancers on areas exposed to sunlight and, in some cases, neurological abnormalities. Some XP-B patients present features of Cockayne syndrome, including dwarfism, sensorineural deafness, microcephaly, mental retardation, pigmentary retinopathy, ataxia, decreased nerve conduction velocities.
    Defects in ERCC3 are a cause of trichothiodystrophy photosensitive (TTDP) [MIM:601675]. TTDP is an autosomal recessive disease characterized by sulfur-deficient brittle hair and nails, ichthyosis, mental retardation, impaired sexual development, abnormal facies and cutaneous photosensitivity correlated with a nucleotide excision repair (NER) defect. Neonates with trichothiodystrophy and ichthyosis are usually born with a collodion membrane. The severity of the ichthyosis after the membrane is shed is variable, ranging from a mild to severe lamellar ichthyotic phenotype. There are no reports of skin cancer associated with TTDP.
  • Sequence similaritiesBelongs to the helicase family. RAD25/XPB subfamily.
    Contains 1 helicase ATP-binding domain.
    Contains 1 helicase C-terminal domain.
  • Cellular localizationNucleus.
  • Information by UniProt

Recombinant Human XPB protein images

  • ab114795 on 12.5% SDS-PAGE Stained with Coomassie Blue.

References for Recombinant Human XPB protein (ab114795)

ab114795 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"