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Rabbit Anti-PYGL  antibody (bs-5011R)  
~~~促銷代碼KT202411~~~
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說明書: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
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產(chǎn)品編號 bs-5011R
英文名稱 Rabbit Anti-PYGL  antibody
中文名稱 肝糖原磷酸化酶抗體
別    名 Glycogen phosphorylase liver; Glycogen phosphorylase liver form; GSD6 antibody Hers disease, glycogen storage disease type VI; Phosphorylase glycogen liver; PYGL_HUMAN.  
研究領(lǐng)域 腫瘤  細(xì)胞生物  免疫學(xué)  糖蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human,Mouse,Rat,Rabbit,Pig,Sheep,Cow,Dog,Horse)
產(chǎn)品應(yīng)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.
理論分子量 97kDa
細(xì)胞定位 細(xì)胞漿 細(xì)胞外基質(zhì) 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PYGL: 451-550/847 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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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