Rabbit Polyclonal Antibody to PYGL

包装: 1G|100G|500G|1KG|100KG

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别名:GSD6
应用:WB,IHC
反应种属:Human, Mouse, Rat
规格：50μl/100μl

Description
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.

Description

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.

Specification
Aliases	GSD6
Swissprot	P06737
WB Predicted band size	97 kDa
Host/Isotype	Rabbit IgG
Storage	Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species Reactivity	Human, Mouse, Rat
Immunogen	Fusion protein of human PYGL
Formulation	pH7.4 PBS, 0.05% NaN3, 40% Glycerol

Application
WB	1/500-1/2000
IHC	1/50-1/200
ELISA	1/5000-1/10000

	Gel: 6%SDS-PAGE, Lysate: 40 μg, Lane 1-2: Mouse liver tissue, 293T cell lysates, Primary antibody: P01686(PYGL Antibody) at dilution 1/400, Secondary antibody: Goat anti rabbit IgG at 1/5000 dilution, Exposure time: 10 seconds
	The image is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using P01686(PYGL Antibody) at dilution 1/50. (Original magnification: ×200)
	The image is immunohistochemistry of paraffin-embedded Human tonsil tissue using P01686(PYGL Antibody) at dilution 1/50. (Original magnification: ×200)