产品名称
PYGM Rabbit Polyclonal Antibody
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
https://www.uniprot.org/uniprot/5837
Human Swissprot No.
P11217
Human Swissprot Link
https://www.uniprot.org/uniprotkb/P11217/entry
Mouse Gene Link
https://www.uniprot.org/uniprot/19309
Mouse Swissprot No.
Q9WUB3
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/Q9WUB3
Rat Swissprot Link
https://www.uniprot.org/uniprotkb/P09812
免疫原
Synthesized peptide derived from human PYGM AA range: 426-476
特异性
This antibody detects endogenous levels of PYGM at Human/Mouse/Rat
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
细胞定位
cytoplasm,cytosol,extracellular exosome,
功能
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.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.