97久久久精品综合88久久_亚洲国产精品一_久热热国产久热_97操操操_北条麻妃在线免费观看_精品国自产拍天天拍

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  關于我們  聯系我們
久久一日本道色综合久久大香,国产在线综合网,蜜桃av免费看
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-ERCC8/PE-Cy3 Conjugated antibody (bs-13095R-PE-Cy3)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-13095R-PE-Cy3
英文名稱 Rabbit Anti-ERCC8/PE-Cy3 Conjugated antibody
中文名稱 PE-Cy3標記的科凱恩氏綜合癥相關蛋白/早衰蛋白CSA抗體
別    名 CKN1; Cockayne syndrome type A; Cockayne syndrome WD repeat protein CSA; CSA; DNA excision repair protein ERCC-8; DNA excision repair protein ERCC8; ERCC 8; ERCC8; ERCC8_HUMAN; excision repair cross-complementing rodent repair deficiency, complementation group 8.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  神經生物學  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat,  (predicted: Horse, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 44kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ERCC8/CSA
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 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 background:
Nucleotide excision repair of DNA lesions occurs more rapidly and at a higher frequency on the template, or the transcribed, strand of DNA and to a much lesser extent on the coding, or the non-transcribed, strand or on transcriptionally inactive DNA. CSA and CSB are two related genes that are responsible for directing this preferential DNA repair pattern, known as transcriptional-repair coupling. Cells from patients with the UV-sensitive nucleotide excision repair disorder Cockayne's syndrome (CS) have specific mutations affecting these genes and results in defects of the preferential repair on the transcribed strand of activated genes. CSA is a protein that belongs in the "WD-repeat" family of proteins. CSB, which is also designated excision repair cross-complementing protein-6 (ERCC-6), is the homolog of the yeast Rad26 protein. CSB belongs in the SWI/SNF family of proteins as it contains helicase motifs and ATPase activity.

Function:
Substrate-recognition component of the CSA complex, a DCX (DDB1-CUL4-X-box) E3 ubiquitin-protein ligase complex, involved in transcription-coupled nucleotide excision repair. The CSA complex (DCX(ERCC8) complex) promotes the ubiquitination and subsequent proteasomal degradation of ERCC6 in a UV-dependent manner; ERCC6 degradation is essential for the recovery of RNA synthesis after transcription-coupled repair. It is required for the recruitement of XAB2, HMGN1 and TCEA1/TFIIS to a transcription-coupled repair complex which removes RNA polymerase II-blocking lesions from the transcribed strand of active genes.

Subunit:
Part of the CSA complex (DCX(ERCC8) complex), a DCX E3 ubiquitin-protein ligase complex containing ERCC8, RBX1, DDB1 and CUL4A; the CSA complex interacts with RNA polymerase II; upon UV irradiation it interacts with the COP9 signalosome and preferentially with the hyperphosphorylated form of RNA polymerase II. Interacts with ERCC6 and KIAA1530/UVSSA. Interacts with a subunit of RNA polymerase II TFIIH. Interacts with DDB1.

Subcellular Location:
Nucleus.

DISEASE:
Defects in ERCC8 are the cause of Cockayne syndrome type A (CSA) [MIM:216400]. Cockayne syndrome is a rare disorder characterized by cutaneous sensitivity to sunlight, abnormal and slow growth, cachectic dwarfism, progeroid appearance, progressive pigmentary retinopathy and sensorineural deafness. There is delayed neural development and severe progressive neurologic degeneration resulting in mental retardation. Two clinical forms are recognized: in the classical form or Cockayne syndrome type 1, the symptoms are progressive and typically become apparent within the first few years or life; the less common Cockayne syndrome type 2 is characterized by more severe symptoms that manifest prenatally. Cockayne syndrome shows some overlap with certain forms of xeroderma pigmentosum. Unlike xeroderma pigmentosum, patients with Cockayne syndrome do not manifest increased freckling and other pigmentation abnormalities in the skin and have no significant increase in skin cancer.

Similarity:
Contains 5 WD repeats.

Database links:

Entrez Gene: 1161 Human

Entrez Gene: 71991 Mouse

Omim: 609412 Human

SwissProt: Q13216 Human

SwissProt: Q8CFD5 Mouse

Unigene: 435237 Human

Unigene: 212208 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權所有 2004-2026 www.kastlife.cn 北京博奧森生物技術有限公司
通過國際質量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫療器械-質量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網安備110107000727號
主站蜘蛛池模板: 91剧场在线观看 | 亚洲欧美日韩网站 | 大地资源在线影视播放 | 国产V精品V欧美V日韩V | 国产亚洲专区 | 性高爱久久久久久久久久久dj | 91蜜桃视频在线观看 | 久久久久久国产精品免费免费狐狸 | 亚人在线播放国产中文字幕 | 美女上床啪啪 | 免费人成又黄又爽又色 | 在线看一级毛片 | 在线三级av | 国产无遮挡色视频免费观看性色 | 日韩精品无码久久一区二区三 | 久久精品成人一区二区三区 | 黄色一级日本 | 四虎永久免费观看在线 | 夜夜春亚洲嫩草一区二区 | 久久免费播放视频 | 午夜天堂精品 | 3344成人免费高清免费视频 | 亚洲曰本AV在线天堂 | 一级做a爰片久久毛片一 | 狠狠操网址 | 福利视频三区 | 亚洲欧美日韩国产专区一区 | 亚洲最新不卡一区二区三区 | 青天衙门高清第一部免费观看 | 欧美1区2区3 | 男女性色大片免费网站 | 精品少妇爆乳无码av无码专区 | 亚洲成人综合网站 | 麻豆视频一区二区三区 | 亚洲人亚洲人色久 | 欧美多人乱大交xxxxx | 成人在线播放网站 | 久久成人免费精品网站 | 亚洲成人伊人 | 91在线观看视频 | 国产一级强片在线观看av |