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

Chromosome: 14; NC_000014.8 (51371935..51411248, complement). Location: 14q21-q22

Store all reagents at 2-8 degree C

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Small volumes of PYGL 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.

MBS021096 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Glycogen Phosphorylase, Liver (PYGL) 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 PYGL. The ELISA analytical biochemical technique of the MBS021096 kit is based on PYGL antibody-PYGL antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect PYGL antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, PYGL. 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(%).

97,149 Da

phosphorylase, glycogen, liver

glycogen phosphorylase, liver form

Glycogen phosphorylase, liver form

PYGL_HUMAN

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; Transferase; EC 2.4.1.1

Chromosomal Location of Human Ortholog: 14q21-q22

Cellular Component: cytoplasm; plasma membrane; cytosol

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

Biological Process: glycogen metabolic process; glycogen catabolic process; carbohydrate metabolic process; glucose metabolic process; 5-phosphoribose 1-diphosphate biosynthetic process; pathogenesis; glucose homeostasis

Disease: Glycogen Storage Disease Vi

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