MTM1 抗体 (AA 225-275) (AbBy Fluor® 594)
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- 抗原 See all MTM1 抗体
- MTM1 (Myotubularin 1 (MTM1))
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抗原表位
- AA 225-275
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适用
- 人, 小鼠, 大鼠
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This MTM1 antibody is conjugated to AbBy Fluor® 594
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应用范围
- Western Blotting (WB), Flow Cytometry (FACS), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- 交叉反应
- 人, 小鼠, 大鼠
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human MTM1/Myotubularin
- 亚型
- IgG
- Top Product
- Discover our top product MTM1 Primary Antibody
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- 应用备注
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FCM 1:20-100
IF(IHC-P) 1:50-200 - 限制
- 仅限研究用
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- 状态
- Liquid
- 浓度
- 1 μg/μL
- 缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- 储存液
- ProClin
- 注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- 储存条件
- -20 °C
- 储存方法
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- 有效期
- 12 months
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- 抗原
- MTM1 (Myotubularin 1 (MTM1))
- 别名
- MTM1 (MTM1 产品)
- 别名
- CNM antibody, MTMX antibody, XLMTM antibody, wu:fb19c01 antibody, zgc:123266 antibody, myotubularin 1 antibody, myotubularin 1 S homeolog antibody, MTM1 antibody, mtm1 antibody, mtm1.S antibody, Mtm1 antibody
- 背景
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Synonyms: CG2, CNM, KIAA4176, mKIAA4176, Mtm, Mtm1, MTM1_HUMAN, MTMX, Myotubular myopathy 1, Myotubularin, XLMTM.
Background: 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.
- 基因ID
- 4534
- UniProt
- Q13496
- 途径
- Inositol Metabolic Process, Skeletal Muscle Fiber Development
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