PYGM rabbit pAb

catalytic activity:(1,4-alpha-D-glucosyl)(n) + phosphate = (1,4-alpha-D-glucosyl)(n-1) + alpha-D-glucose 1-phosphate.,cofactor:Pyridoxal phosphate.,disease:Defects in PYGM are the cause of glycogen storage disease type 5 (GSD5) [MIM:232600]; also known as McArdle disease. GSD5 is a metabolic disorder resulting in myopathy characterized by exercise intolerance, cramps, muscle weakness and recurrent myoglobinuria.,enzyme regulation:Activity of phosphorylase is controlled both by allosteric means (through the noncovalent binding of metabolites) and by covalent modification. Thus AMP allosterically activates, whereas ATP, ADP, and glucose-6-phosphate allosterically inhibit, phosphorylase B.,function: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.,PTM:Phosphorylation of Ser-15 converts phosphorylase B (unphosphorylated) to phosphorylase A.,similarity:Belongs to the glycogen phosphorylase family.,subunit:Homodimer. Dimers associate into a tetramer to form the enzymatically active phosphorylase A.,