FITC标记的科凯恩氏综合症相关蛋白/早衰蛋白CSA抗体上海沪震实业有限公司

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产品名称: FITC标记的科凯恩氏综合症相关蛋白/早衰蛋白CSA抗体
英文名称: Anti-ERCC8/FITC
抗体货号: HZ-13095R-FITC
产品规格: 100ul
级    别: 分析纯, , 分析纯,
产品产地: 中国/上海
品牌商标: HZbscience
价    格: 2980元
抗原: Rabbit
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: 
FITCconjugate:FITC
应用范围: ICC=1:50-200 IF=1:50-200
更新时间: 2018/9/14 10:09:00
详细资料:  实验方法技术资料
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使用范围:仅限科研使用,不能应用于临床
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产品详细描述

 Rabbit Anti-ERCC8/FITC Conjugated antibody

FITC标记的科凯恩氏综合症相关蛋白/早衰蛋白CSA抗体

 

英文名称 Anti-ERCC8/FITC
中文名称 FITC标记的科凯恩氏综合症相关蛋白/早衰蛋白CSA抗体
别    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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  神经生物学  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Horse, 
产品应用 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    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ERCC8/CSA
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
产品介绍 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
   
   

DNA损伤的核苷酸切除修复在模板或转录的DNA链上以更快和更高频率发生,而在编码或非转录的DNA链上或在转录失活的DNA上发生的频率要低得多。CSA和CSB是两个相关的基因,负责指导这种优先的DNA修复模式,称为转录-修复耦合。来自UV敏感核苷酸切除修复障碍性Cockayne综合征(CS)患者的细胞具有影响这些基因的特异性突变,导致激活基因转录链的优先修复缺陷。CsA是属于蛋白质“WD重复”家族的蛋白质。CSB是酵母RAD26蛋白的同源物,它也被称为切除修复交叉互补蛋白-6(ErCC-6)。CSB属于蛋白质的SWI/SNF家族,因为它含有解旋酶基序和ATP酶活性。

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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