肌肉糖原磷酸化酶抗体

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英文名称  Anti-PYGM 

中文名称  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. 

浓    度  1mg/1ml 

规 格  0.2ml/200μg

抗体来源  Rabbit  

克隆类型  polyclonal 

交叉反应  Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep

肌肉糖原磷酸化酶抗体的多样性：

抗体的异质性。抗体的组成极为复杂，是由成千上万、多种多样的免疫球蛋白（Ig）分子所组成。这些Ig分子在形状、大小、结构以及氨基酸的组成和排列上，既相似，又有差别。由于有差别，它们的电泳活性就有很大的变化。

因为抗体具有与抗原决定簇相对应的结合部位（抗原结合簇），所以抗体与抗原的结合具有特异性。另一方面，抗体本身是一种蛋白质，具有本身的氨基酸组成、排列和立体结构，对异种动物来说，它又是抗原。各类Ig都具有可用血清学方法检出的抗原特异性，它们表现出不同的血清学类型。

肌肉糖原磷酸化酶抗体的相关产品:

SMEK2/PP4R3B  丝氨酸/苏氨酸蛋白磷酸酶3B抗体 0.2ml

SM-MHC  心肌肌球蛋白抗体（α-myosin） 0.1ml

SMMHC/Myosin  平滑肌肌球蛋白重链抗体 0.1ml

SMN1  运动神经元生存蛋白1 0.1ml   

产品类型  一抗    

研究领域  肿瘤 细胞生物 免疫学 转录调节因子 糖蛋白  

蛋白分子量  predicted molecular weight: 97kDa 

性    状  Lyophilized or Liquid 

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 PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide 

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:100-500 

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

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. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

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. 

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 肌肉糖原磷酸化酶抗体disorder resulting in myopathy characterized by exercise intolerance, cramps, muscle weakness and recurrent myoglobinuria. 

Similarity : Belongs to the glycogen phosphorylase family.