Immunogen
Purified recombinant human TFIIH p89 (C-terminus) protein fragments expressed in E.coli.
Specificity
TFIIH p89 Monoclonal Antibody detects endogenous levels of TFIIH p89 protein.
Formulation
Purified mouse monoclonal in buffer containing 0.1M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.2% sodium azide, 50% glycerol.
Dilution rate
Western Blot: 1/1000 - 1/2000. Not yet tested in other applications.
Purification process (Immunogen)
Affinity purification
Background
This gene encodes an ATP-dependent DNA helicase that functions in nucleotide excision repair. The encoded protein is a subunit of basal transcription factor 2 (TFIIH) and, therefore, also functions in class II transcription. Mutations in this gene are associated with Xeroderma pigmentosum B, Cockayne's syndrome, and trichothiodystrophy. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2014],
Function
disease: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.,disease:Defects 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). Xeroder
Protein name
TFIIH basal transcription factor complex helicase XPB subunit
Other name
ERCC3; XPB; XPBC; TFIIH basal transcription factor complex helicase XPB subunit; Basic transcription factor 2 89 kDa subunit; BTF2 p89; DNA excision repair protein ERCC-3; DNA repair protein complementing XP-B cells; TFIIH basal transcripti
Fields
>>Basal transcription factors;>>Nucleotide excision repair
Human protein sequence Database
P19447
Mouse protein sequence database
P49135
Rat protein sequence database
Q4G005
Cellular localization
Nucleus.
Tissue expression
Adipose tissue,Epithelium,Placenta,
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