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MTM1 抗体 (AA 225-275)

MTM1 适用: 人, 小鼠, 大鼠 WB, FACS, IF (p), IHC (p) 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN1386965
发货至: 中国
  • 抗原 See all MTM1 抗体
    MTM1 (Myotubularin 1 (MTM1))
    抗原表位
    • 15
    • 8
    • 4
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 225-275
    适用
    • 43
    • 37
    • 24
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    人, 小鼠, 大鼠
    宿主
    • 41
    • 3
    • 1
    克隆类型
    • 43
    • 2
    多克隆
    标记
    • 19
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This MTM1 antibody is un-conjugated
    应用范围
    • 39
    • 15
    • 13
    • 12
    • 5
    • 4
    • 1
    • 1
    • 1
    • 1
    Western Blotting (WB), Flow Cytometry (FACS), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (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
  • 应用备注
    WB 1:300-5000
    FCM 1:20-100
    IHC-P 1:200-400
    IF(IHC-P) 1:50-200
    限制
    仅限研究用
  • 状态
    Liquid
    浓度
    1 μg/μL
    缓冲液
    0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % 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
    4 °C,-20 °C
    储存方法
    Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
    有效期
    12 months
  • 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

    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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