TFIIH p89 Monoclonal Antibody
- 货号:YM1106
- 应用:WB
- 种属:Human;Mouse;Rat;Bovine;Dog
- 简介:
- >>Basal transcription factors;>>Nucleotide excision repair
- 蛋白名称:
- TFIIH basal transcription factor complex helicase XPB subunit
- 免疫原:
- Purified recombinant human TFIIH p89 (C-terminus) protein fragments expressed in E.coli.
- 特异性:
- TFIIH p89 Monoclonal Antibody detects endogenous levels of TFIIH p89 protein.
- 组成:
- Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- 稀释:
- WB 1:1000 - 1:2000. Not yet tested in other applications.
- 纯化工艺:
- Affinity purification
- 储存:
- -15°C to -25°C/1 year(Do not lower than -25°C)
- 其他名称:
- 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
- 背景:
- 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],
- 功能:
- 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
- 组织表达:
- Adipose tissue,Epithelium,Placenta,
- Western Blot analysis using TFIIH p89 Monoclonal Antibody against PC12, HepG2 cell lysate.