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Rabbit Anti-PYGM/BF594 Conjugated antibody (bs-5012R-BF594)
訂購熱線:400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-5012R-BF594
英文名稱 Rabbit Anti-PYGM/BF594 Conjugated antibody
中文名稱 BF594標記的肌肉糖原磷酸化酶抗體
別    名 Glycogen phosphorylase muscle form; Muscpho; Myophosphorylase; Phosphorylase glycogen muscle (McArdle syndrome glycogen storage disease type V); PYGM_HUMAN.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  免疫學  轉錄調節因子  糖蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Rat,  (predicted: Human, Mouse, Dog, Pig, Cow, Horse, Sheep, )
產品應用 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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PYGM
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 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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