Summary: 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].

glycogen phosphorylase LMIM:613741Ensembl:ENSG00000100504HGNC:HGNC:9725PA3406814q22.1

Gene Categories:


GO terms in PYGL

Term TypeEvidence TypeGO Term IDGO Des.
CCTASGO:0005576extracellular region
CCTASGO:0034774secretory granule lumen
CCHDAGO:0070062extracellular exosome
CCTASGO:1904813ficolin-1-rich granule lumen
BPIMPGO:0005977glycogen metabolic process
BPIBAGO:0005980glycogen catabolic process
BPTASGO:0005980glycogen catabolic process
BPIEAGO:00060155-phosphoribose 1-diphosphate biosynthetic process
BPIMPGO:0042593glucose homeostasis
BPTASGO:0043312neutrophil degranulation
BPIEAGO:0070266necroptotic process
MFIDAGO:0002060purine nucleobase binding
MFIPIGO:0005515protein binding
MFIDAGO:0005524ATP binding
MFNASGO:0005536glucose binding
MFIDAGO:0008144drug binding
MFEXPGO:0008184glycogen phosphorylase activity
MFIBAGO:0008184glycogen phosphorylase activity
MFIDAGO:0008184glycogen phosphorylase activity
MFIMPGO:0008184glycogen phosphorylase activity
MFIDAGO:0016208AMP binding
MFIDAGO:0019842vitamin binding
MFIBAGO:0030170pyridoxal phosphate binding
MFIDAGO:0032052bile acid binding
MFNASGO:0042803protein homodimerization activity
MFIEAGO:0102250linear malto-oligosaccharide phosphorylase activity
MFIEAGO:0102499SHG alpha-glucan phosphorylase activity

Gene expression in normal tissue: PYGL

Gene-model tissue-cancer distribution: Bubble Plot

Gene-drug pathway distribution

Pathways in PYGL

DatabasePathway IDPathway Des.
reactomeR-HSA-168249Innate Immune System
reactomeR-HSA-168256Immune System
reactomeR-HSA-6798695Neutrophil degranulation
reactomeR-HSA-70221Glycogen breakdown (glycogenolysis)
reactomeR-HSA-71387Metabolism of carbohydrates
reactomeR-HSA-8982491Glycogen metabolism
wikipathwaysWP231TNF alpha Signaling Pathway
wikipathwaysWP500Glycogen Metabolism
smpdbSMP00058Starch and Sucrose Metabolism
smpdbSMP00552Glycogen synthetase deficiency
smpdbSMP00553Glycogenosis, Type III. Cori disease, Debrancher glycogenosis
smpdbSMP00554Glycogenosis, Type IV. Amylopectinosis, Anderson disease
smpdbSMP00555Glycogenosis, Type VI. Hers disease
smpdbSMP00556Mucopolysaccharidosis VI. Sly syndrome
smpdbSMP00557Sucrase-isomaltase deficiency
kegghsa00500Starch and sucrose metabolism - Homo sapiens (human)
kegghsa04217Necroptosis - Homo sapiens (human)
kegghsa04910Insulin signaling pathway - Homo sapiens (human)
kegghsa04922Glucagon signaling pathway - Homo sapiens (human)
kegghsa04931Insulin resistance - Homo sapiens (human)

Gene-Drug: Aster Plot

Drug IDDrug NameModel Num.

Gene in drug-gene network: Network Plot

Gene-drug targets distribution

Gene Structure: PDB

Models in PYGL