Product Name	Anti-PYGL/AF594
Chinese Name	AF594标记的肝糖原磷酸化酶抗体
Alias	Glycogen phosphorylase liver; Glycogen phosphorylase liver form; GSD6 antibody Hers disease, glycogen storage disease type VI; Phosphorylase glycogen liver; PYGL_HUMAN.
Research Area	Tumour  Cell biology  immunology  glycoprotein
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species
Applications	ICC=1:50-200 IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	97kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human PYGL
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Product Detail	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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