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Rabbit Anti-PYGL/FITC Conjugated antibody
background:
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.
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.
Subunit:
Homodimer. Dimers associate into a tetramer to form the enzymatically active phosphorylase A. Interacts with PPP1R3B
Post-translational modifications:
Phosphorylation of Ser-15 converts phosphorylase B (unphosphorylated) to phosphorylase A.
DISEASE:
Defects in PYGL are the cause of glycogen storage disease type 6 (GSD6) [MIM:232700]. A metabolic disorder characterized by mild to moderate hypoglycemia, mild ketosis, growth retardation, and prominent hepatomegaly. Heart and skeletal muscle are not affected.
Similarity:
Belongs to the glycogen phosphorylase family.
Database links:
Entrez Gene: 5836 Human
Omim: 232700 Human
SwissProt: P06737 Human
Unigene: 282417 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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