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    GATM Rabbit pAb (bs-13296R)  
    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
    50ul/1180.00元
    100ul/1980.00元
    200ul/2800.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-13296R
    英文名稱(chēng) GATM Rabbit pAb
    中文名稱(chēng) GATM蛋白抗體
    別    名 AGAT; AT; GATM; GATM_HUMAN; Glycine amidinotransferase; Glycine amidinotransferase, mitochondrial; L-arginine:glycine amidinotransferase; mitochondrial; Transamidinase.  
    研究領(lǐng)域 腫瘤  細胞生物  發(fā)育生物學(xué)  神經(jīng)生物學(xué)  信號轉導  新陳代謝  線(xiàn)粒體  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    克 隆 號
    交叉反應 (predicted: Human,Mouse,Rat,Pig,Sheep,Zebrafish,Dog,Cat)
    產(chǎn)品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 44 kDa
    檢測分子量
    細胞定位 細胞漿 細胞膜 線(xiàn)粒體
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human GATM: 141-240/423 
    亞    型 IgG
    純化方法 affinity purified by Protein A
    緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    PubMed PubMed
    產(chǎn)品介紹 Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development. May be involved in the response to heart failure by elevating local creatine synthesis.

    Function:
    Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development. May be involved in the response to heart failure by elevating local creatine synthesis.

    Subunit:
    Homodimer. There is an equilibrium between the monomeric and dimeric forms, shifted towards the side of the monomer.

    Subcellular Location:
    Mitochondrion inner membrane. Cytoplasm. The mitochondrial form is found in the intermembrane space probably attached to the outer side of the inner membrane.

    Tissue Specificity:
    Expressed in brain, heart, kidney, liver, lung, salivary gland and skeletal muscle tissue, with the highest expression in kidney. Biallelically expressed in placenta and fetal tissues.

    DISEASE:
    Defects in GATM are the cause of arginine:glycine amidinotransferase deficiency (AGAT deficiency) [MIM:612718]. AGAT deficiency is an autosomal recessive disorder characterized by developmental delay/regression, mental retardation, severe disturbance of expressive and cognitive speech, and severe depletion of creatine/phosphocreatine in the brain.

    Similarity:
    Belongs to the amidinotransferase family.

    SWISS:
    P50440

    Gene ID:
    2628

    Database links:

    Entrez Gene: 2628 Human

    Entrez Gene: 67092 Mouse

    Entrez Gene: 81660 Rat

    Entrez Gene: 266799 Zebrafish

    Omim: 602360 Human

    SwissProt: P50440 Human

    SwissProt: Q9D964 Mouse

    SwissProt: P50442 Rat

    SwissProt: Q6PH19 Zebrafish



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