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Rabbit Anti-PYGM/RBITC Conjugated antibody (bs-5012R-RBITC)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-5012R-RBITC
英文名称1 Rabbit Anti-PYGM/RBITC Conjugated antibody
中文名称 罗丹明(RBITC)标记的肌肉糖原磷酸化酶抗体
别    名 Glycogen phosphorylase muscle form; Muscpho; Myophosphorylase; Phosphorylase glycogen muscle (McArdle syndrome glycogen storage disease type V); PYGM_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  细胞生物  免疫学  转录调节因子  糖蛋白  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Rat,  (predicted: Human, Mouse, Dog, Pig, Cow, Horse, Sheep, )
产品应用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 97kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PYGM
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
产品介绍 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.

Post-translational modifications:
Phosphorylation of Ser-15 converts phosphorylase B (unphosphorylated) to phosphorylase A.

DISEASE:
Defects in PYGM are the cause of glycogen storage disease type 5 (GSD5) [MIM:232600]; also known as McArdle disease. GSD5 is a metabolic disorder resulting in myopathy characterized by exercise intolerance, cramps, muscle weakness and recurrent myoglobinuria.

Similarity:
Belongs to the glycogen phosphorylase family.

Database links:

Entrez Gene: 5837 Human

Entrez Gene: 19309 Mouse

Entrez Gene: 24701 Rat

Omim: 608455 Human

SwissProt: P11217 Human

SwissProt: Q9WUB3 Mouse

SwissProt: P09812 Rat

Unigene: 154084 Human

Unigene: 27806 Mouse

Unigene: 11238 Rat



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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