Anti-MTM1 antibody produced in rabbit

半岛bd体育手机客户端 说明

一般描述

MTM1 (myotubularin 1) is a lipid phosphatase, which is abundant at the junctional SR (sarcoplasmic reticulum) of the muscle triads. It is a member of conserved phosphatase family myotubularin, which contains both active phosphatases and inactive myotubularin-related proteins (MTMRs).

免疫原

Myotubularin recombinant protein epitope signature tag (PrEST)

应用

Anti-MTM1 antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project (www.proteinatlas.org). Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
Applications in which this antibody has been used successfully, and the associated peer-reviewed papers, are given below.
Immunofluorescence (1 paper)

生化/生理作用

MTM1 (myotubularin 1) protein plays an essential role in facilitating the curvature of SR (sarcoplasmic reticulum) membrane and SR remodelling. Therefore, it is critical for homeostasis of calcium and excitation-contraction coupling. The major function of this protein is the dephosphorylation of phosphatidylinositol-3-phosphate (PtdIns3P) and phosphatidylinositol-3,5-bisphosphate (PtdIns(3,5P)₂). Mutations in this gene are linked to X-linked myotubular myopathy (XLMTM), which is an inherited disorder characterized by severe generalized hypotonia and skeletal muscles weakness with respiratory insufficiency. Mostly this disease is fatal in the initial months of life. Heterozygous mutations resulting in abnormal splicing in this gene are associated with early onset, slowly progressive myopathy. c.431delT (p.Leu144fs) mutation in this gene results in bilateral subdural hygromas and cephalhaematomas in male twins suffering from severe myotubular myopathy.

特点和优势

Prestige Antibodies^?are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies^?to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:

• IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
• Protein array of 364 human recombinant protein fragments.

联系

Corresponding Antigen APREST74421.

外形

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide

法律信息

Prestige Antibodies is a registered trademark of sigma-aldrich Co. LLC

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

基本信息

人类蛋白质图谱编号

HPA010008 Human Protein Atlas characterization data

半岛bd体育手机客户端 性质

生物来源	rabbit
质量水平	100
偶联物	unconjugated
抗体形式	affinity isolated antibody
antibody product type	primary antibodies
克隆	polyclonal
半岛bd体育手机客户端 线	Prestige Antibodies?Powered by Atlas Antibodies
形式	buffered aqueous glycerol solution
species reactivity	human
包装	antibody small pack of 25 μL
technique(s)	immunofluorescence: 0.25-2 μg/mL immunohistochemistry: 1:500-1:1000
免疫原序列	KFNVDGWTVYNPVEEYRRQGLPNHHWRITFINKCYELCDTYPALLVVPYRASDDDLRRVATFRSRNRIPVLSWIHPENKTVIVRCSQPLVGMSGKRNKDDEKYLDVIRETNKQISKLTIYDARPSVNAVANK
UniProt登记号	Q13496
运输	wet ice
储存温度	?20℃
Gene Information	human ... MTM1(4534)

安全信息

储存分类代码	10 - Combustible liquids
WGK	WGK 1
闪点(F)	Not applicable
闪点(C)	Not applicable
个人防护装备	Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)