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
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About This Item
Productos recomendados
origen biológico
rabbit
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
Condiciones de envío
wet ice
temp. de almacenamiento
−20°C
modificación del objetivo postraduccional
unmodified
Información sobre el gen
human ... MTM1(4534)
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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)
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:
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.
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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)
Certificados de análisis (COA)
Busque Certificados de análisis (COA) introduciendo el número de lote del producto. Los números de lote se encuentran en la etiqueta del producto después de las palabras «Lot» o «Batch»
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Encuentre la documentación para los productos que ha comprado recientemente en la Biblioteca de documentos.
Vandana A Gupta et al.
PLoS genetics, 9(6), e1003583-e1003583 (2013-07-03)
X-linked myotubular myopathy (XLMTM) is a congenital disorder caused by mutations of the myotubularin gene, MTM1. Myotubularin belongs to a large family of conserved lipid phosphatases that include both catalytically active and inactive myotubularin-related proteins (i.e., "MTMRs"). Biochemically, catalytically inactive
Jorge Oliveira et al.
European journal of human genetics : EJHG, 21(5), 540-549 (2012-09-13)
Myotubular myopathy (MIM#310400), the X-linked form of Centronuclear myopathy (CNM) is mainly characterized by neonatal hypotonia and inability to maintain unassisted respiration. The MTM1 gene, responsible for this disease, encodes myotubularin - a lipidic phosphatase involved in vesicle trafficking regulation
Ji Hyun Jeon et al.
Yonsei medical journal, 52(3), 547-550 (2011-04-14)
X-linked myotubular myopathy (XLMTM) is a rare congenital muscle disorder, caused by mutations in the MTM1 gene. Affected male infants present severe hypotonia, and generalized muscle weakness, and the disorder is most often complicated by respiratory failure. Herein, we describe
Valérie Tosch et al.
Neuromuscular disorders : NMD, 20(6), 375-381 (2010-05-04)
X-linked centronuclear myopathy (XLMTM), also called myotubular myopathy, is a severe congenital myopathy characterized by generalized hypotonia and weakness at birth and the typical histological finding of centralization of myo-nuclei. It is caused by mutations in the MTM1 gene encoding
Bilateral subdural hygromas and cephalhaematomas in male twins with severe myotubular myopathy caused by a Novel c.431delT (p.Leu144fs) mutation in MTM1 gene.
Tejasvi Chaudhari et al.
Journal of paediatrics and child health, 47(1-2), 64-65 (2010-05-27)
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