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Description for Protein PYGL

phosphorylase, glycogen, liver
2 total interacting proteins; 1 platelet interacting proteins
Icon Book Platelet Evidence (proteome studies/others : 4/0)
(membrane: 1; microparticles: 1; platelet: 1; secretome: 2)
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 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. [provided by RefSeq] (PubMed Links)
Domains and Motifs:
None Available
Gene Ontology:
Gene Ontology Annotations
KEGG - Enzyme ID(s):
2.4.1.1
KEGG - Orthology:
K00688
KEGG - Pathway(s):
hsa00500; hsa04910
(The yellow boxes represents platelet proteins)
Nomenclature / Alternative Names:
Liver glycogen phosphorylase; HLGP; EC 2.4.1.1
Approved Symbol:
PYGL
(De-) Phosphorylations:
Total (de-) phosphorylation sites: 7
Human (de-) phosphorylation sites: 7; No platelet phosphorylation sites
Phosphorylation Targets:
Total phosphorylation targets: 0
Human phosphorylation targets: 0;Predicted platelet targets: 0
Protein Characteristics:
Isoform-specific Information
Icon DrugsAssociated Drugs (DrugBank Accession):

  • 1-N-Acetyl-Beta-D-Glucosamine(db);
  • 1-{2-[3-(2-Chloro-4,5-difluoro-benzoyl)-ureido]-4-fluoro-phenyl}-piperidine-4-carboxylic acid(db);
  • 2-Methyl-2,4-Pentanediol(db);
  • 4-[3-(2-Chloro-4,5-difluoro-benzoyl)ureido]-3-trifluoromethoxybenzoic acid(db);
  • 5-Chloro-1h-Indole-2-Carboxylic Acid{[Cyclopentyl-(2-Hydroxy-Ethyl)-Carbamoyl]-Methyl}-Amide(db);
  • 5-chloro-N-{4-[(1R)-1,2-dihydroxyethyl]phenyl}-1H-indole-2-carboxamide(db);
  • 7,9-Dihydro-1h-Purine-2,6,8(3h)-Trione(db);
  • Adenosine monophosphate(db);
  • Beta-D-Glucose(db);
  • CP-526423(db);
  • Cp403700, (S)-1-{2-[(5-Chloro-1h-Indole-2-Carbonyl)-Amino]-3-Phenyl-Propionyl}-Azetidine-3-Carboxylate(db);
  • N-(2-CHLORO-4-FLUOROBENZOYL)-N'-(5-HYDROXY-2-METHOXYPHENYL)UREA(db);
  • Phosphonoserine(db);
  • Pyridoxal Phosphate(db)

    Associated Genetic Diseases:

  • Glycogen storage disease VI(Pd)
    		• Predicted Transmembrane Domains:
  • Isoform 1 : 0
    • Additional Identifiers:

    HPRD: 01987 Entrez Gene ID: 5836 OMIM ID: 232700 Swissprot Accession: P06737;