Anti-GMPPB antibody produced in rabbit

产品说明

一般描述

GMPPB (guanosine diphosphate mannose (GDP-mannose) pyrophosphorylase B) protein is a part of the glycosylation pathway. It was first identified and partially characterized from Athrobacter sp. and is highly conserved among multiple species. This protein is composed of 360 amino acids, and shows high sequence similarity with porcine GMPPB. This protein resides in the cytoplasm, and has a human paralog called GMPPA. Both these proteins share 30% identity. This gene is localized to human chromosome 3p21.31.

免疫原

Mannose-1-phosphate guanyltransferase beta recombinant protein epitope signature tag (PrEST)

应用

All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project (www.proteinatlas.org)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. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.

生化/生理作用

GMPPB (guanosine diphosphate mannose (GDP-mannose) pyrophosphorylase B) catalyzes the conversion of mannose-1-phosphate to GDP-mannose, in the presence of GTP. It is responsible for the O-mannosylation of multiple proteins such as, α-dystroglycan (α-DG). Mutations in this gene lead to the aggregation of GMPPB in the cytosol and in proximity to membrane protrusions. It also results in hypoglycosylation of α-DG, leading to congenital and limb-girdle muscular dystrophies. Mutations in this gene are linked to generalized epilepsy, as glycosylation might play an important part in the neuronal channels and network. Mutations in this gene are as linked to isolated episodes of rhabdomyolysis. This gene locus is also linked to musculo-eye-brain disorders.

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 APREST70273.

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.

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