anti-Laforin antibody
CAT.NO. : ARG42011
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概述
产品描述 | Rabbit Polyclonal antibody recognizes Laforin |
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反应物种 | Hu, Ms, Rat |
应用 | WB |
宿主 | Rabbit |
克隆 | Polyclonal |
同位型 | IgG |
靶点名称 | Laforin |
抗原物种 | Human |
抗原 | Recombinant fusion protein corresponding to aa. 244-331 of Human Laforin (NP_005661.1). |
偶联标记 | Un-conjugated |
別名 | Laforin; EPM2; Glucan phosphatase; EC 3.1.3.16; EC 3.1.3.-; Lafora PTPase; LAFPTPase; EC 3.1.3.48; MELF |
应用说明
应用建议 |
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应用说明 | * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. | ||||
阳性对照 | HeLa | ||||
实际分子量 | ~ 35 kDa |
属性
形式 | Liquid |
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纯化 | Affinity purified. |
缓冲液 | PBS (pH 7.3), 0.02% Sodium azide and 50% Glycerol. |
抗菌剂 | 0.02% Sodium azide |
稳定剂 | 50% Glycerol |
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. 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 | |
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基因名称 | EPM2A |
全名 | epilepsy, progressive myoclonus type 2A, Lafora disease (laforin) |
背景介绍 | This gene encodes a dual-specificity phosphatase that associates with polyribosomes. The encoded protein may be involved in the regulation of glycogen metabolism. Mutations in this gene have been associated with myoclonic epilepsy of Lafora. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2008] |
生物功能 | Has both dual-specificity protein phosphatase and glucan phosphatase activities. Together with the E3 ubiquitin ligase NHLRC1/malin, appears to be involved in the clearance of toxic polyglucosan and protein aggregates via multiple pathways. Dephosphorylates phosphotyrosine, phosphoserine and phosphothreonine substrates in vitro. Has also been shown to dephosphorylate MAPT. Shows strong phosphatase activity towards complex carbohydrates in vitro, avoiding glycogen hyperphosphorylation which is associated with reduced branching and formation of insoluble aggregates. Forms a complex with NHLRC1/malin and HSP70, which suppresses the cellular toxicity of misfolded proteins by promoting their degradation through the ubiquitin-proteasome system (UPS). Acts as a scaffold protein to facilitate PPP1R3C/PTG ubiquitination by NHLRC1/malin. Also promotes proteasome-independent protein degradation through the macroautophagy pathway. Isoform 2, an inactive phosphatase, could function as a dominant-negative regulator for the phosphatase activity of isoform 1. [UniProt] |
细胞定位 | Cytoplasm. Note=Under glycogenolytic conditions localizes to the nucleus. [UniProt] |
预测分子量 | 37 kDa |
翻译后修饰 | Polyubiquitinated by NHLRC1/malin. Phosphorylation on Ser-25 by AMPK affects the phosphatase activity of the enzyme and its ability to homodimerize and interact with NHLRC1, PPP1R3C or PRKAA2. [UniProt] |
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