Anti-PYGL monoclonal antibody Made to order

Antibody Isotype

IgG

Species Reactivity

Human

Immunogen

A synthetic peptide of human PYGL is used for rabbit immunization.

Conjugate

Alternative Names

PYGL; phosphorylase, glycogen, liver; phosphorylase, glycogen; liver; glycogen phosphorylase, liver form; glycogen storage disease type VI; Hers disease; GSD6;

Entrez Gene ID

UniProt ID

Antigen Description

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 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, or 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.

Pathway

Glucose metabolism, organism-specific biosystem; Glycogen Metabolism, organism-specific biosystem; Glycogen breakdown (glycogenolysis), organism-specific biosystem; Insulin signaling pathway, organism-specific biosystem; Insulin signaling pathway, conserved biosystem; Metabolism, organism-specific biosystem; Metabolism of carbohydrates, organism-specific biosystem.