catalog number :
MBS7603208
products full name :
PYGL Rabbit Polyclonal
products short name :
[PYGL]
products name syn :
[GSD6, phosphorylase, glycogen, liver, PYGL]
other names :
[PYGL protein, partial; Glycogen phosphorylase, liver form; glycogen phosphorylase, liver form; phosphorylase, glycogen, liver]
products gene name :
[PYGL]
other gene names :
[PYGL; PYGL; GSD6]
uniprot entry name :
PYGL_HUMAN
reactivity :
Human, Mouse, Rat
specificity :
Human, Mouse ,Rat; other species are not tested. Please decide the specificity by homology
purity :
>=95% as determined by SDS-PAGE. Immunogen Affinity Purified
storage stability :
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)
tested application :
ELISA (EIA), Immunohistochemistry (IHC), Western Blot (WB), Immunoprecipitation (IP), Immunofluorescence (IF)
app notes :
WB : 1:500-1:3000. IP :1:500-1:5000. IHC : 1:20-1:200. IF : 1:20-1:200
image1 heading :
Immunohistochemistry
image2 heading :
SDS-PAGE
other info1 :
Immunogen: Phosphorylase, glycogen, liver
products description :
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.
ncbi acc num :
AAH65011.1
ncbi pathways :
Glucagon Signaling Pathway (1144996); Glucagon Signaling Pathway (1158201); Glucose Metabolism Pathway (1269959); Glycogen Metabolism Pathway (198856); Glycogen Breakdown (glycogenolysis) Pathway (1269963); Insulin Resistance Pathway (1272486); Insulin Signaling Pathway (83090); Insulin Signaling Pathway (501); Metabolic Pathways (132956); Metabolism Pathway (1269956)
ncbi summary :
This gene encodes a homodimeric protein that catalyses the cleavage of alpha-1,4-glucosidic bonds to release glucose-1-phosphate from liver glycogen stores. This protein switches from inactive phosphorylase B to active phosphorylase A by phosphorylation of serine residue 15. Activity of this enzyme is further regulated by multiple allosteric effectors and hormonal controls. Humans have three glycogen phosphorylase genes that encode distinct isozymes that are primarily expressed in liver, brain and muscle, respectively. The liver isozyme serves the glycemic demands of the body in general while the brain and muscle isozymes supply just those tissues. In glycogen storage disease type VI, also known as Hers disease, mutations in liver glycogen phosphorylase inhibit the conversion of glycogen to glucose and results in moderate hypoglycemia, mild ketosis, growth retardation and hepatomegaly. Alternative splicing results in multiple transcript variants encoding different isoforms.[provided by RefSeq, Feb 2011]
uniprot summary :
PYGL: 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. Defects in PYGL are the cause of glycogen storage disease type 6 (GSD6). A metabolic disorder characterized by mild to moderate hypoglycemia, mild ketosis, growth retardation, and prominent hepatomegaly. Heart and skeletal muscle are not affected. Belongs to the glycogen phosphorylase family. Protein type: EC 2.4.1.1; Carbohydrate Metabolism - starch and sucrose; Transferase. Chromosomal Location of Human Ortholog: 14q21-q22. Cellular Component: cytoplasm; cytosol; plasma membrane. Molecular Function: AMP binding; ATP binding; bile acid binding; drug binding; glucose binding; glycogen phosphorylase activity; protein binding; protein homodimerization activity; purine binding; pyridoxal phosphate binding; vitamin binding. Biological Process: 5-phosphoribose 1-diphosphate biosynthetic process; glucose homeostasis; glycogen catabolic process; glycogen metabolic process. Disease: Glycogen Storage Disease Vi