產(chǎn)品編號 | bs-5012R-BF350 |
英文名稱1 | Rabbit Anti-PYGM/BF350 Conjugated antibody |
中文名稱 | BF350標(biāo)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(guī)格價格 | 100ul/2980元 購買 大包裝/詢價 |
說 明 書 | 100ul |
研究領(lǐng)域 | 腫瘤 細(xì)胞生物 免疫學(xué) 轉(zhuǎn)錄調(diào)節(jié)因子 糖蛋白 |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | Rat, (predicted: Human, Mouse, Dog, Pig, Cow, Horse, Sheep, ) |
產(chǎn)品應(yīng)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(chǎn)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 Omim: 608455 Human SwissProt: P11217 Human SwissProt: Q9WUB3 Mouse 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. |