產(chǎn)品編號(hào) | bs-23502R-BF594 |
英文名稱1 | Rabbit Anti-PGAM2/BF594 Conjugated antibody |
中文名稱 | BF594標(biāo)記的PGAM2抗體 |
別 名 | BPG dependent PGAM 2; BPG-dependent PGAM 2; GSD10; MGC88743; Muscle specific phosphoglycerate mutase; Muscle-specific phosphoglycerate mutase; OTTHUMP00000207787; PGAM 2; PGAM M; PGAM-M; Pgam2; PGAM2_HUMAN; PGAMM. |
規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買 大包裝/詢價(jià) |
說(shuō) 明 書(shū) | 100ul |
研究領(lǐng)域 | |
抗體來(lái)源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, ) |
產(chǎn)品應(yīng)用 | ICC=1:50-200 IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
分 子 量 | 29kDa |
性 狀 | Lyophilized or Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human PGAM2 |
亞 型 | IgG |
純化方法 | affinity purified by Protein A |
儲(chǔ) 存 液 | Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4. |
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: Phosphoglycerate mutase (PGAM) catalyzes the reversible reaction of 3-phosphoglycerate (3-PGA) to 2-phosphoglycerate (2-PGA) in the glycolytic pathway. The PGAM is a dimeric enzyme containing, in different tissues, different proportions of a slow-migrating muscle (MM) isozyme, a fast-migrating brain (BB) isozyme, and a hybrid form (MB). This gene encodes muscle-specific PGAM subunit. Mutations in this gene cause muscle phosphoglycerate mutase eficiency, also known as glycogen storage disease X. [provided by RefSeq, Sep 2009] Function: Interconversion of 3- and 2-phosphoglycerate with 2,3-bisphosphoglycerate as the primer of the reaction. Can also catalyze the reaction of EC 5.4.2.4 (synthase) and EC 3.1.3.13 (phosphatase), but with a reduced activity. Tissue Specificity: In mammalian tissues there are two types of phosphoglycerate mutase isozymes: type-M in muscles and type-B in other tissues. DISEASE: Defects in PGAM2 are the cause of glycogen storage disease type 10 (GSD10) [MIM:261670]. A metabolic disorder characterized by myoglobinuria, increased serum creatine kinase levels, decreased phosphoglycerate mutase activity, myalgia, muscle pain, muscle cramps and excercise intolerance. Similarity: Belongs to the phosphoglycerate mutase family. BPG-dependent PGAM subfamily. Database links: Entrez Gene: 5224 Human Entrez Gene: 56012 Mouse Omim: 612931 Human SwissProt: P15259 Human SwissProt: O70250 Mouse Unigene: 23217 Cow Unigene: 632642 Human Unigene: 219627 Mouse Unigene: 9738 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |