ARG65221
anti-LARGE1 antibody
anti-LARGE1 antibody for Western blot and Human
Cell Biology and Cellular Response antibody
概述
产品描述 | Goat Polyclonal antibody recognizes LARGE1 |
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反应物种 | Hu |
预测物种 | Cow, Dog |
应用 | WB |
特异性 | Reported variants represent identical protein: NP_004728.1, NP_598397.1 |
宿主 | Goat |
克隆 | Polyclonal |
同位型 | IgG |
靶点名称 | LARGE1 |
抗原物种 | Human |
抗原 | C-SEADVNSENLQKQ |
偶联标记 | Un-conjugated |
別名 | EC 2.4.1.-; MDC1D; EC 2.4.-.-; Acetylglucosaminyltransferase-like 1A; MDDGA6; MDDGB6; Glycosyltransferase-like protein LARGE1; EC 2.4.2.- |
应用说明
应用建议 |
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应用说明 | WB: Recommend incubate at RT for 1h. * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. |
属性
形式 | Liquid |
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纯化 | Purified from goat serum by antigen affinity chromatography. |
缓冲液 | Tris saline (pH 7.3), 0.02% Sodium azide and 0.5% BSA. |
抗菌剂 | 0.02% Sodium azide |
稳定剂 | 0.5% BSA |
浓度 | 0.5 mg/ml |
存放说明 | For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C or below. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. |
注意事项 | For laboratory research only, not for drug, diagnostic or other use. |
生物信息
数据库连接 |
Swiss-port # O95461 Human Glycosyltransferase-like protein LARGE1 |
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基因名称 | LARGE1 |
全名 | LARGE xylosyl- and glucuronyltransferase 1 |
背景介绍 | This gene, which is one of the largest in the human genome, encodes a member of the N-acetylglucosaminyltransferase gene family. It encodes a glycosyltransferase which participates in glycosylation of alpha-dystroglycan, and may carry out the synthesis of glycoprotein and glycosphingolipid sugar chains. It may also be involved in the addition of a repeated disaccharide unit. Mutations in this gene cause MDC1D, a novel form of congenital muscular dystrophy with severe mental retardation and abnormal glycosylation of alpha-dystroglycan. Alternative splicing of this gene results in two transcript variants that encode the same protein. [provided by RefSeq, Jul 2008] |
生物功能 | Bifunctional glycosyltransferase with both xylosyltransferase and beta-1,3-glucuronyltransferase activities involved in the biosynthesis of the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1) (PubMed:22223806). Phosphorylated O-mannosyl trisaccharid is required for binding laminin G-like domain-containing extracellular proteins with high affinity and plays a key role in skeletal muscle function and regeneration. LARGE elongates the glucuronyl-beta-1,4-xylose-beta disaccharide primer structure initiated by B3GNT1/B4GAT1 by adding repeating units [-3-Xylose-alpha-1,3-GlcA-beta-1-] to produce a heteropolysaccharide (PubMed:25279699). [UniProt] |
研究领域 | Cell Biology and Cellular Response antibody |
预测分子量 | 88 kDa |
检测图片 (1) Click the Picture to Zoom In