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The glycogen phosphorylase monomer is a large protein, composed of 842 amino acids with a mass of 97.434 kDa in muscle cells. While the enzyme can exist as an inactive monomer or tetramer, it is biologically active as a dimer of two identical subunits.
Myophosphorylase or glycogen phosphorylase, muscle associated (PYGM) is the muscle isoform of the enzyme glycogen phosphorylase and is encoded by the PYGM gene. This enzyme helps break down glycogen (a form of stored carbohydrate) into glucose-1-phosphate (not glucose), so it can be used within the muscle cell.
Glycogen phosphorylase, liver form (PYGL), also known as human liver glycogen phosphorylase (HLGP), is an enzyme that in humans is encoded by the PYGL gene on chromosome 14. [1] [2] 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.
The PYGB gene encodes one of three major glycogen phosphorylase isoforms, which are distinguished by their different structures and subcellular localizations: brain (PYGB), muscle (PYGM), and liver . [2] [3] GPBB is the longest of the three isozymes, with a length of 862 residues, due to the extended 3'-UTR at the enzyme's C-terminal.
Glycogen synthase (UDP-glucose-glycogen glucosyltransferase) is a key enzyme in glycogenesis, the conversion of glucose into glycogen. It is a glycosyltransferase ( EC 2.4.1.11 ) that catalyses the reaction of UDP-glucose and (1,4- α - D -glucosyl) n to yield UDP and (1,4- α - D -glucosyl) n+1 .
A key regulator of PP1 is glycogen phosphorylase a, which serves as a glucose sensor in hepatocytes. [12] When glucose levels are low, phosphorylase a in its active R state has PP1 bound tightly. This binding to phosphorylase a prevents any phosphatase activity of PP1 and maintains the glycogen phosphorylase in its active phosphorylated ...
Two autosomal recessive forms of this disease occur, childhood-onset and adult-onset. The gene for myophosphorylase, PYGM (the muscle-type of the glycogen phosphorylase gene), is located on chromosome 11q13. According to the most recent publications, 95 different mutations have been reported.
phosphorylate glycogen phosphorylase via phosphorylase kinase (activating it) [12] phosphorylate Acetyl-CoA carboxylase (inhibiting it) inhibit glycogenesis. phosphorylate glycogen synthase (inhibiting it) [12] stimulate glycolysis. phosphorylate phosphofructokinase 2 (stimulating it, cardiomyocytes only) myocyte (cardiac muscle) cardiovascular