|Other Names||Glycogen phosphorylase, muscle form, Myophosphorylase, PYGM|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP1450b was selected from the C-term region of human PYGM. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|Format||The synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml deionized water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|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.|
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Provided below are standard protocols that you may find useful for product applications.
PYGM catalyzes and regulates the breakdown of glycogen to glucose-1-phosphate. Defects in PYGM are the cause of glycogen storage disease type 5 (GSD5), also known as McArdle disease. GSD5 is a metabolic disorder resulting in myopathy characterized by exercise intolerance, cramps, muscle weakness and recurrent myoglobinuria.
Tsoi, S.C., et al., J. Soc. Gynecol. Investig. 10(8):496-502 (2003).Bruno, C., et al., Neuromuscul. Disord. 12(5):498-500 (2002).Hadjigeorgiou, G.M., et al., Neuromuscul. Disord. 12(9):824-827 (2002).Deschauer, M., et al., Mol. Genet. Metab. 74(4):489-491 (2001).Kubisch, C., et al., Hum. Mutat. 12(1):27-32 (1998).
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