Search Thermo Fisher Scientific
图: 1 / 3
Antibody detects endogenous levels of PYGL only when phosphorylated at Ser15.
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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
蛋白别名: glycogen liver; Glycogen phosphorylase, liver form; GPLL; liver glycogen phosphorylase
基因别名: GSD6; Lgp; PYGL
UniProt ID: (Human) P06737, (Mouse) Q9ET01, (Rat) P09811
Entrez Gene ID: (Human) 5836, (Mouse) 110095, (Rat) 64035