NP_001157412.1
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NCBI GenBank Nucleotide #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
93,134 Da
NCBI Official Full Name
glycogen phosphorylase, liver form isoform 2
NCBI Official Synonym Full Names
phosphorylase, glycogen, liver
NCBI Official Synonym Symbols
NCBI Protein Information
glycogen phosphorylase, liver form
UniProt Protein Name
Glycogen phosphorylase, liver form
UniProt Entry Name
PYGL_HUMAN
NCBI Summary for GP- II
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]
UniProt Comments for GP- II
PYGL: Phosphorylase is an important allosteric enzyme in carbohydrate metabolism. Enzymes from different sources differ in their regulatory mechanisms and in their natural substrates. However, all known phosphorylases share catalytic and structural properties. Defects in PYGL are the cause of glycogen storage disease type 6 (GSD6). A metabolic disorder characterized by mild to moderate hypoglycemia, mild ketosis, growth retardation, and prominent hepatomegaly. Heart and skeletal muscle are not affected. Belongs to the glycogen phosphorylase family.
Protein type: EC 2.4.1.1; Transferase; Carbohydrate Metabolism - starch and sucrose
Chromosomal Location of Human Ortholog: 14q21-q22
Cellular Component: cytoplasm; plasma membrane; cytosol
Molecular Function: glycogen phosphorylase activity; protein homodimerization activity; bile acid binding; purine binding; drug binding; vitamin binding; ATP binding; AMP binding; pyridoxal phosphate binding; glucose binding
Biological Process: glycogen metabolic process; glycogen catabolic process; carbohydrate metabolic process; 5-phosphoribose 1-diphosphate biosynthetic process; glucose metabolic process; pathogenesis; glucose homeostasis
Disease: Glycogen Storage Disease Vi
Research Articles on GP- II
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Products associated with GP- II elisa kit
Pathways associated with GP- II elisa kit
Diseases associated with GP- II elisa kit
Organs/Tissues associated with GP- II elisa kit
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