| 產(chǎn)品編號 | bs-13095R-BF350 |
| 英文名稱1 | Rabbit Anti-ERCC8/BF350 Conjugated antibody |
| 中文名稱 | BF350標(biāo)記的科凱恩氏綜合癥相關(guān)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(guī)格價(jià)格 | 100ul/2980元 購買 大包裝/詢價(jià) |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 細(xì)胞生物 神經(jīng)生物學(xué) 表觀遺傳學(xué) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Human, Mouse, Rat, (predicted: Horse, ) |
| 產(chǎn)品應(yīng)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(chǎn)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. |
