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Anti-MTM1 Polyclonal Antibody

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货号 TMAB-09068

Anti-MTM1 Polyclonal Antibody 是一种 Rabbit 抗体,靶向 MTM1。Anti-MTM1 Polyclonal Antibody 可用于 WB。

Anti-MTM1 Polyclonal Antibody

Anti-MTM1 Polyclonal Antibody

一键复制产品信息
Rating icon 还可以
货号 TMAB-09068

Anti-MTM1 Polyclonal Antibody 是一种 Rabbit 抗体,靶向 MTM1。Anti-MTM1 Polyclonal Antibody 可用于 WB。

规格价格库存数量
50 μL
¥ 1,160
5日内发货
100 μL
¥ 1,960
5日内发货
200 μL
¥ 2,795
5日内发货
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产品介绍


生物活性
产品描述
Anti-MTM1 Polyclonal Antibody is a Rabbit antibody targeting MTM1. Anti-MTM1 Polyclonal Antibody can be used in WB.
Ig Type
IgG
反应种属
Mouse (predicted:Human,Rat,Dog,Pig,Rabbit)
应用WB
推荐剂量
WB: 1:500-2000
抗体种类
Polyclonal
宿主来源Rabbit
亚细胞定位Cytoplasm. Cell membrane; Peripheral membrane protein. Cell projection, filopodium. Cell projection, ruffle. Late endosome. Note=Localizes as a dense cytoplasmic network. Also localizes to the plasma membrane, including plasma membrane extensions such as filopodia and ruffles. Predominantly located in the cytoplasm following interaction with MTMR12. Recruited to the late endosome following EGF stimulation. [DOMAIN] The GRAM domain mediates binding to PI(3,5)P2 and, with lower affinity, to other phosphoinositides.
构建方式Hybridoma Polyclonal Antibody
纯化方式Protein A purified
性状Liquid
缓冲液0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
浓度1mg/ml
研究背景X-linked recessive myotubular myopathy is a congenital muscular disease characterized by severe hypotonia and generalized muscle weakness that, in most cases, leads to early postnatal death. The gene responsible for myotubular myopathy MTM1 encodes a dual specificity phosphatase, named myotubularin, which is highly conserved through evolution. The gene for MTM1 is localized to a 300 kb critical region on human Xq128 between IDS and GRBRA3. Human MTM1, a 603 amino-acid protein, is mutated in myotubular myopathy. The largely related protein hMTMR2 is found mutated in a recessive form of Charcot-Marie-Tooth neuropathy. Myotubularin is primarily a lipid phosphatase that acts on phosphatidylinositol 3-monophosphate and is involved in the regulation of the phosphatidylinositol 3-kinase (PI3-kinase) pathway and membrane trafficking. Wild-type myotubularin can directly dephosphorylate PI3P and PI4P in vitro. Thus, it decreases PI3P levels by down-regulating PI3K activity and by facilitating the degradation of PI3P.
抗原信息
免疫原
KLH conjugated synthetic peptide: human MTM1/Myotubularin
抗原种属
Human
基因名称
MTM1
基因ID
蛋白名称
Myotubularin
Uniprot ID
功能
Lipid phosphatase which dephosphorylates phosphatidylinositol 3-monophosphate (PI3P) and phosphatidylinositol 3,5-bisphosphate (PI(3,5)P2). Has also been shown to dephosphorylate phosphotyrosine- and phosphoserine-containing peptides. Negatively regulates EGFR degradation through regulation of EGFR trafficking from the late endosome to the lysosome. Plays a role in vacuolar formation and morphology. Regulates desmin intermediate filament assembly and architecture. Plays a role in mitochondrial morphology and positioning. Required for skeletal muscle maintenance but not for myogenesis.
化学信息
分子量Theoretical: 70 kDa.
储存&溶解度
储存方式Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles.
运输方式Shipping with blue ice.
存储store at low temperature | -20°C for 1 year

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