For Research Use Only. Not for use in diagnostic procedures.

This assay has high sensitivity and excellent specificity for detection of Human PYGL. No significant cross-reactivity or interference between Human PYGL and analogues was observed.

Store at 2-8 degree C.

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Small volumes of GP elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS281879 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Glycogen phosphorylase (GP) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing GP. The ELISA analytical biochemical technique of the MBS281879 kit is based on GP antibody-GP antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect GP antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, GP. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Principle of the Assay: This assay employs a two-site sandwich ELISA to quantitate PYGL in samples. An antibody specific for PYGL has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyPYGL present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for PYGL is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of PYGL bound in the initial step. The color development is stopped and the intensity of the color is measured.

93,134 Da

glycogen phosphorylase L

glycogen phosphorylase, liver form

Glycogen phosphorylase, liver form

This gene encodes a homodimeric protein that catalyses the cleavage of alpha-1,4-glucosidic bonds to release glucose-1-phosphate from liver glycogen stores. This protein switches from inactive phosphorylase B to active phosphorylase A by phosphorylation of serine residue 15. Activity of this enzyme is further regulated by multiple allosteric effectors and hormonal controls. Humans have three glycogen phosphorylase genes that encode distinct isozymes that are primarily expressed in liver, brain and muscle, respectively. The liver isozyme serves the glycemic demands of the body in general while the brain and muscle isozymes supply just those tissues. In glycogen storage disease type VI, also known as Hers disease, mutations in liver glycogen phosphorylase inhibit the conversion of glycogen to glucose and results in moderate hypoglycemia, mild ketosis, growth retardation and hepatomegaly. Alternative splicing results in multiple transcript variants encoding different isoforms.[provided by RefSeq, Feb 2011]

PYGL: Phosphorylase is an important allosteric enzyme in carbohydrate metabolism. Enzymes from different sources differ in their regulatory mechanisms and in their natural substrates. However, all known phosphorylases share catalytic and structural properties. Defects in PYGL are the cause of glycogen storage disease type 6 (GSD6). A metabolic disorder characterized by mild to moderate hypoglycemia, mild ketosis, growth retardation, and prominent hepatomegaly. Heart and skeletal muscle are not affected. Belongs to the glycogen phosphorylase family.

Protein type: Carbohydrate Metabolism - starch and sucrose; EC 2.4.1.1; Transferase

Chromosomal Location of Human Ortholog: 14q22.1

Cellular Component: cytoplasm; cytosol; extracellular exosome; extracellular region; ficolin-1-rich granule lumen; secretory granule lumen

Molecular Function: AMP binding; ATP binding; bile acid binding; drug binding; glucose binding; glycogen phosphorylase activity; linear malto-oligosaccharide phosphorylase activity; protein binding; protein homodimerization activity; purine binding; pyridoxal phosphate binding; vitamin binding

Biological Process: 5-phosphoribose 1-diphosphate biosynthetic process; glucose homeostasis; glycogen catabolic process; glycogen metabolic process; necroptotic process; neutrophil degranulation

Disease: Glycogen Storage Disease Vi

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