产品详情
简单介绍：
Anti-MTM1抗体产品质量稳定，实验效果明显，货期快，价格优惠，欢迎垂询订购！我公司长期供应**组化抗体、WB抗体、**组化试剂盒和抗体试验所需全部相关试剂、荧光标记抗体、单克隆抗体、多克隆抗体、各种标记的二抗IgG/IgM/IgD/IgA等科研实验抗体。Anti-MTM1抗体用于**组化实验,WB实验,相应的标记抗体有HRP标记抗体,FITC标记,BIO等。
详情介绍：
Rabbit Anti-MTM1
Cat. Number:
Anti-MTM1抗体KL-9178R
Quantity size:
0.2ml
Concentration:
1mg/ml Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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
Anti-MTM1抗体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.
Also known as:
CG2; CNM; KIAA4176; mKIAA4176;
Anti-MTM1抗体Mtm; Mtm1; MTM1_HUMAN; MTMX; Myotubular myopathy 1; Myotubularin; XLMTM.
Specificity:
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Rabbit Polyclonal IgG, affinity purified by Protein A.
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Reacts with: Human,
Anti-MTM1抗体Mouse, Rat, Dog, Pig, Rabbit, .
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Immunogen: KLH conjugated synthetic peptide derived from human MTM1/Myotubularin.
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Predicted Molecular Weight: 70kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200
Not yet tested in other applications. Anti-MTM1抗体
Optimal working dilutions must be determined by the end user.
Not yet tested in other applications. Anti-MTM1抗体
Optimal working dilutions must be determined by the end user.
