產(chǎn)品編號(hào) | bs-13461R-BF594 |
英文名稱 | Rabbit Anti-GMPPB/BF594 Conjugated antibody |
中文名稱 | BF594標(biāo)記的GDP甘露糖焦磷酸化酶B抗體 |
別 名 | GDP mannose pyrophosphorylase B; GDP-mannose pyrophosphorylase B; GMPPB; GMPPB_HUMAN; GTP-mannose-1-phosphate guanylyltransferase beta; KIAA1851; Mannose 1 phosphate guanylyltransferase; Mannose-1-phosphate guanyltransferase beta. |
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 信號(hào)轉(zhuǎn)導(dǎo) 表觀遺傳學(xué) |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | |
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 | 40kDa |
性 狀 | Lyophilized or Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human GMPPB |
亞 型 | IgG |
純化方法 | affinity purified by Protein A |
儲(chǔ) 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: GMPPB is a 360 amino acid protein that belongs to the transferase hexapeptide repeat family and is involved in protein modification pathways. Functioning as a GDP-mannose pyrophosphorylase, GMPPB enzymatically catalyzes the conversion of mannose-1-phosphate and GTP to GDP-mannose and a free phosphate, a reaction that is involved in the production of N-linked oligosaccharides. Defects in the gene encoding GMPPB that cause errors in the glycosylation pathway may lead to congenital disorders of glycosylation (CDG). CDGs are multisystemic diseases that often involve both the central and peripheral nervous systems and are often characterized by endocrine and coagulation disorders. GMPPB is expressed as two isoforms due to alternative splicing events. Subcellular Location: Belongs to the transferase hexapeptide repeat family. Similarity: Belongs to the transferase hexapeptide repeat family. Database links: UniProtKB/Swiss-Prot: Q9Y5P6.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |