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Rabbit Anti-PYGL  antibody (bs-5011R)
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-5011R
英文名称 PYGL
中文名称 肝糖原磷酸化酶抗体
别    名 Glycogen phosphorylase liver; Glycogen phosphorylase liver form; GSD6 antibody Hers disease, glycogen storage disease type VI; Phosphorylase glycogen liver; PYGL_HUMAN.  
研究领域 肿瘤  细胞生物  免疫学  糖蛋白  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human,Mouse,Rat,Dog,Pig,Cow,Horse,Rabbit,Sheep)
产品应用 IHC-P=1:100-500, IHC-F=1:100-500, IF=1:100-500, ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 97kDa
细胞定位 细胞浆 细胞外基质 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PYGL: 451-550/847 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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.

SWISS:
P06737

Gene ID:
5836

Database links:

Entrez Gene: 5836 Human

Omim: 232700 Human

SwissProt: P06737 Human

Unigene: 282417 Human



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