以下为您提供的是肌肉糖原磷酸化酶抗体的说明书,了解更多关于肌肉糖原磷酸化酶抗体的英文名称,肌肉糖原磷酸化酶抗体别名,肌肉糖原磷酸化酶抗体规格,请看以下详细介绍.
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.
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