MTM1 抗体 (AA 225-275) (AbBy Fluor® 680)
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北京 101111
Quick Overview for MTM1 抗体 (AA 225-275) (AbBy Fluor® 680) (ABIN5007106)
抗原
See all MTM1 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 225-275
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交叉反应
- 人, 小鼠, 大鼠
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纯化方法
- Purified by Protein A.
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免疫原
- KLH conjugated synthetic peptide derived from human MTM1/Myotubularin
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亚型
- IgG
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anti-Myotubularin 1 (MTM1) (AA 1-100) antibody
MTM1 适用: 人 WB, ELISA, IHC (p), RNAi 宿主: 小鼠 Monoclonal 1C10 unconjugated
anti-Myotubularin 1 (MTM1) (C-Term) antibodyVerified MTM1 适用: 人, 小鼠 WB, ELISA, IHC 宿主: 山羊 Polyclonal unconjugated
anti-Myotubularin 1 (MTM1) (AA 1-603) antibodyMTM1 适用: 人 WB 宿主: 兔 Polyclonal unconjugated
anti-Myotubularin 1 (MTM1) (AA 225-275) antibodyMTM1 适用: 人, 小鼠, 大鼠 WB, FACS, IF (p), IHC (p) 宿主: 兔 Polyclonal unconjugated
anti-Myotubularin 1 (MTM1) (AA 484-603) antibodyMTM1 适用: 人 WB, ELISA, IHC 宿主: 兔 Polyclonal unconjugated
anti-Myotubularin 1 (MTM1) (C-Term) antibodyMTM1 适用: 人, 小鼠, 大鼠 WB, ELISA, IHC 宿主: 兔 Polyclonal unconjugated
anti-Myotubularin 1 (MTM1) (AA 1-603) antibodyMTM1 适用: 人 WB 宿主: 小鼠 Polyclonal unconjugated
anti-Myotubularin 1 (MTM1) (AA 1-30), (N-Term) antibodyMTM1 适用: 人 WB, IHC (p) 宿主: 兔 Polyclonal RB0722 unconjugated
anti-Myotubularin 1 (MTM1) (AA 566-594), (C-Term) antibodyMTM1 适用: 人, 小鼠 WB 宿主: 兔 Polyclonal RB0856 unconjugated
anti-Myotubularin 1 (MTM1) (AA 451-485) antibodyMTM1 适用: 斑马鱼 WB 宿主: 兔 Polyclonal RB52010 unconjugated
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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
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浓度
- 1 μg/μL
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缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
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储存液
- ProClin
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注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
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有效期
- 12 months
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- MTM1 (Myotubularin 1 (MTM1))
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别名
- MTM1
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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.
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基因ID
- 4534
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UniProt
- Q13496
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途径
- Inositol Metabolic Process, Skeletal Muscle Fiber Development
抗原
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