Thermo Fisher Scientific PYGL Polyclonal Antibody

Applications

Product Specifications

Target Information

This gene encodes a homodimeric protein that catalyzes 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 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 (Hers disease), mutations in liver glycogen phosphorylase inhibit glycogen conversion to glucose, resulting in moderate hypoglycemia, mild ketosis, growth retardation, and hepatomegaly. Alternative splicing results in multiple transcript variants encoding different isoforms.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.

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