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DNA損傷修復酶ERCC3蛋白抗體
  • 產(chǎn)品貨號：
    BN41008R
  • 中文名稱：
    DNA損傷修復酶ERCC3蛋白抗體
  • 英文名稱：
    Rabbit anti-XPB Polyclonal antibody
  • 品牌：
    Biorigin

  • 貨號

    產(chǎn)品規(guī)格

    售價

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  • BN41008R-100ul

    100ul

    ￥2360.00

    交叉反應(yīng)：Human,Rat(predicted:Mouse,Chicken,Dog,Pig,Cow,Horse,Rabbit) 推薦應(yīng)用：IHC-P,IHC-F,IF,ELISA

  • BN41008R-200ul

    200ul

    ￥3490.00

    交叉反應(yīng)：Human,Rat(predicted:Mouse,Chicken,Dog,Pig,Cow,Horse,Rabbit) 推薦應(yīng)用：IHC-P,IHC-F,IF,ELISA

產(chǎn)品描述

英文名稱XPB
中文名稱DNA損傷修復酶ERCC3蛋白抗體
別    名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 89 kDa subunit; TFIIH antibody TFIIH basal transcription factor complex 89 kDa subunit; TFIIH basal transcription factor complex helicase XPB subunit; TFIIH p89; Xeroderma pigmentosum group B-complementing protein.  
研究領(lǐng)域腫瘤  細胞生物  免疫學  染色質(zhì)和核信號  表觀遺傳學  
抗體來源Rabbit
克隆類型Polyclonal
交叉反應(yīng)Human, Rat,  (predicted: Mouse, Chicken, Dog, Pig, Cow, Horse, Rabbit, )
產(chǎn)品應(yīng)用ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量89kDa
細胞定位細胞核 
性    狀Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human XPB/ERCC3:601-700/782 
亞    型IgG
純化方法affinity purified by Protein A
儲 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產(chǎn)品介紹Initiation of transcription from protein-coding genes in eukaryotes is a complex process that requires RNA polymerase II, as well as families of basal transcription factors. Binding of the factor TFIID (TBP) to the TATA box is believed to be the first step in the formation of a multiprotein complex containing several additional factors, including TFIIA, TFIIB, TFIIE, TFIIF and TFIIH. TFIIH (or BTF2) is a multisubunit transcription/DNA repair factor that possesses several enzymatic activities. The core of TFIIH is composed of 5 subunits, designated p89 (XPB or ERCC3), p62, p52, p44 and p34. Additional subunits of the TFIIH complex are p80 (XPD or ERCC2) and the ternary kinase complex composed of Cdk7, cyclin H and MAT1. Both p89 and p80 have ATP-dependent helicase activity. The p62, p52 and p44 subunits have been shown to be involved in nucleotide excision repair.

Function:
ATP-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.

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). 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.

Similarity:
Belongs to the helicase family. RAD25/XPB subfamily.
Contains 1 helicase ATP-binding domain.
Contains 1 helicase C-terminal domain.

SWISS:
P19447

Gene ID:
2071

Database links:

Entrez Gene: 2071 Human

Entrez Gene: 13872 Mouse

Entrez Gene: 291703 Rat

Entrez Gene: 324323 Zebrafish

Omim: 133510 Human

SwissProt: P19447 Human

SwissProt: P49135 Mouse

SwissProt: Q4G005 Rat

SwissProt: Q7ZVV1 Zebrafish

Unigene: 469872 Human

Unigene: 282335 Mouse

Unigene: 44012 Rat

Unigene: 6574 Zebrafish



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications