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    Rabbit Anti-PGAM2/APC Conjugated antibody (bs-23502R-APC)
    訂購熱線(xiàn):400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術(shù)支持:techsupport@bioss.com.cn
    說(shuō) 明 書(shū): 100ul  
    100ul/2980.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-23502R-APC
    英文名稱(chēng) Rabbit Anti-PGAM2/APC Conjugated antibody
    中文名稱(chēng) APC標記的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.  
    規格價(jià)格 100ul/2980元 購買(mǎi)        大包裝/詢(xún)價(jià)
    說(shuō) 明 書(shū) 100ul  
    研究領(lǐng)域
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
    產(chǎn)品應用 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 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.05M PB, pH 7.5.
    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: 515067 Cow

    Entrez Gene: 5224 Human

    Entrez Gene: 56012 Mouse

    Entrez Gene: 100188980 Pig

    Entrez Gene: 24959 Rat

    Omim: 612931 Human

    SwissProt: Q32KV0 Cow

    SwissProt: P15259 Human

    SwissProt: O70250 Mouse

    SwissProt: P16290 Rat

    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.
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