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Merck

HPA004055

Sigma-Aldrich

Anti-GNPTG antibody produced in rabbit

enhanced validation

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

Sinónimos:

Anti-GlcNAc-1-phosphotransferase subunit γ antibody produced in rabbit, Anti-N-Acetylglucosamine-1-phosphotransferase subunit γ precursor antibody produced in rabbit, Anti-UDP-N-Acetylglucosamine-1-phosphotransferase, subunit γ antibody produced in rabbit

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About This Item

Código UNSPSC:
12352203
Atlas de proteínas humanas número:
NACRES:
NA.41

origen biológico

rabbit

Nivel de calidad

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

validación mejorada

recombinant expression
Learn more about Antibody Enhanced Validation

Inmunógeno

N-Acetylglucosamine-1-phosphotransferase subunit γ precursor recombinant protein epitope signature tag (PrEST)

Aplicación

All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.

The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.

Acciones bioquímicas o fisiológicas

GNPTG (N-acetylglucosamine-1-phosphate transferase, γ subunit) is referred as RJD9, GNPTAG, LP2537 and C16orf27. It encodes the γ-subunit of the GlcNAc-1-phosphotransferase. Mutation in this gene causes an autosomal recessive disease called Mucolipidosis type III gamma (MLIII gamma), characterized by missorting and cellular loss of lysosomal enzymes leading to lysosomal accumulation of storage material. It plays a key role in the transport of lysosomal hydrolases to the lysosome.

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 APREST86806

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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Shuang Liu et al.
Gene, 535(2), 294-298 (2013-12-10)
Mucolipidosis type III gamma (MLIII gamma) is an autosomal recessive disease caused by a mutation in the GNPTG gene, which encodes the γ subunit of the N-acetylglucosamine-1-phosphotransferase (GlcNAc-1-phosphotransferase). This protein plays a key role in the transport of lysosomal hydrolases
Sandra Pohl et al.
Biochimica et biophysica acta, 1792(3), 221-225 (2009-08-27)
The N-Acetylglucosaminyl-1-phosphotransferase plays a key role in the generation of mannose 6-phosphate (M6P) recognition markersessential for efficient transport of lysosomal hydrolases to lysosomes. The phosphotransferase is composed of six subunits (alpha2, beta2, gamma2). The alpha- and beta-subunits are catalytically active
Sandra Pohl et al.
American journal of medical genetics. Part A, 152A(1), 124-132 (2009-12-25)
Mucolipidosis type III gamma (MLIII, pseudo-Hurler polydystrophy) is a rare autosomal recessive disorder where the activity of the multimeric GlcNAc-1-phosphotransferase is reduced and formation of the mannose 6-phosphate (M6P) recognition marker on lysosomal enzymes is impaired. In this disease, the

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