HPA010008

Anti-MTM1 antibody produced in rabbit

Ab1, Prestige Antibodies^® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

• Sinónimos: Anti-Myotubularin antibody produced in rabbit
• Código UNSPSC: 12352203
• Atlas de proteínas humanas número: HPA010008
• NACRES: NA.43

Propiedades

• origen biológico: rabbit
• Nivel de calidad: 100
• conjugado: unconjugated
• forma del anticuerpo: affinity isolated antibody
• tipo de anticuerpo: primary antibodies
• clon: polyclonal
• Línea del producto: Prestige Antibodies^® Powered by Atlas Antibodies
• Formulario: buffered aqueous glycerol solution
• reactividad de especies: human
• técnicas: immunofluorescence: 0.25-2 μg/mL; immunohistochemistry: 1:500-1:1000
• secuencia del inmunógeno: KFNVDGWTVYNPVEEYRRQGLPNHHWRITFINKCYELCDTYPALLVVPYRASDDDLRRVATFRSRNRIPVLSWIHPENKTVIVRCSQPLVGMSGKRNKDDEKYLDVIRETNKQISKLTIYDARPSVNAVANK
• Nº de acceso UniProt: Q13496
• Condiciones de envío: wet ice
• temp. de almacenamiento: −20°C
• modificación del objetivo postraduccional: unmodified
• Información sobre el gen: human ... MTM1(4534)

Descripción

Descripción general

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).

Inmunógeno

Myotubularin recombinant protein epitope signature tag (PrEST)

Aplicación

Anti-MTM1 antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project . 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)

Acciones bioquímicas o fisiológicas

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.

Características y beneficios

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.

Ligadura / enlace

Corresponding Antigen APREST74421

Forma física

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

Información legal

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

Cláusula de descargo de responsabilidad

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.

Información de seguridad

• Código de clase de almacenamiento: 10 - Combustible liquids
• Clase de riesgo para el agua (WGK): WGK 1
• Punto de inflamabilidad (°F): Not applicable
• Punto de inflamabilidad (°C): Not applicable
• Equipo de protección personal: Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)