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Upingbio
SKU:YP-Ab-09040-100UL
PYGM rabbit pAb
PYGM rabbit pAb
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- Gene Name: PYGM
- Immunogen: Synthesized peptide derived from human PYGM AA range: 426-476
- Specificity: This antibody detects endogenous levels of PYGM at Human/Mouse/Rat
- Composition: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- Source: Polyclonal, Rabbit,IgG
- Dilution ratio: WB 1:500-2000
- Purification process: The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
- Concentration: 1 mg/ml
- Storage: -20°C/1 year
- Background: This gene encodes a muscle enzyme involved in glycogenolysis. Highly similar enzymes encoded by different genes are found in liver and brain. Mutations in this gene are associated with McArdle disease (myophosphorylase deficiency), a glycogen storage disease of muscle. Alternative splicing results in multiple transcript variants.[provided by RefSeq, Sep 2009],
- Function: 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 know
- Species: Human; Mouse;Rat
- Range: WB
- Protein: PYGM
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