• Target：
• TFIIH

• Fields：
• >>Basal transcription factors;>>Nucleotide excision repair

• Gene Name：
• ERCC3

• Protein Name：
• TFIIH basal transcription factor complex helicase XPB subunit

• Human Gene Id：
• 2071

• Human Swiss Prot No：
• P19447

• Mouse Gene Id：
• 13872

• Mouse Swiss Prot No：
• P49135

• Rat Gene Id：
• 291703

• Rat Swiss Prot No：
• Q4G005

• 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：
• Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

• Source：
• Monoclonal, Mouse

• Dilution：
• WB 1:1000 - 1:2000. Not yet tested in other applications.

• Purification：
• Affinity purification

• Concentration：
• 1 mg/ml

• Storage Stability：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• 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

• Molecular Weight(Da)：
• 89kD

• 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

• Subcellular Location：
• Nucleus.

• Expression：
• Adipose tissue,Epithelium,Placenta,

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