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    Rabbit Anti-GFPT1/APC Conjugated antibody (bs-13341R-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-13341R-APC
    英文名稱(chēng) Rabbit Anti-GFPT1/APC Conjugated antibody
    中文名稱(chēng) APC標記的谷氨酰胺6-磷酸果糖轉移酶抗體
    別    名 D-fructose-6-phosphate amidotransferase 1; GFA; GFAT 1; GFAT; GFAT1; GFAT1m; GFPT; Gfpt1; GFPT1_HUMAN; Glucosamine--fructose-6-phosphate aminotransferase [isomerizing] 1; Glutamine--fructose-6-phosphate transaminase 1; Glutamine:fructose 6 phosphate amidotransferase 1; Hexosephosphate aminotransferase 1.  
    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 細胞生物  信號轉導  糖尿病  新陳代謝  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應 Human,  (predicted: Mouse, Rat, Chicken, Dog, Pig, 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 79kDa
    性    狀 Lyophilized or Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human GFPT1
    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:
    Glutamine:fructose-6-phosphate amidotransferase (GFAT1) is the first and rate-limiting enzyme for the entry of glucose into the hexosamine biosynthesis pathway (HBP) in mammals. GFAT1, a member of the N-terminal nucleophile class of amidotransferases, converts fructose-6-phosphate into N-acetylglucosamine-6-phosphate. Hyperglycemia-induced insulin resistance, a condition in which exposure to high concentrations of glucose and insulin results in insulin resistance, may result from increased glucose metabolism through the HBP. Hypergylcemia-induced insulin resistance is a characteristic feature of type 2 diabetes. Consequently, GFAT1 is a potential therapeutic target in the treatment of type 2 diabetes.

    Function:
    Controls the flux of glucose into the hexosamine pathway. Most likely involved in regulating the availability of precursors for N- and O-linked glycosylation of proteins.

    Subunit:
    Homotetramer

    Tissue Specificity:
    Isoform 1 is predominantly expressed in skeletal muscle. Not expressed in brain. Seems to be selectively expressed in striated muscle.

    DISEASE:
    Defects in GFPT1 are the cause of limb-girdle myasthenia with tubular aggregates (LGMTA) [MIM:610542]. A congenital myasthenic syndrome characterized by onset of proximal muscle weakness in the first decade. Individuals with this condition have a recognizable pattern of weakness of shoulder and pelvic girdle muscles, and sparing of ocular or facial muscles. EMG classically shows a decremental response to repeated nerve stimulation, a sign of neuromuscular junction dysfunction. Affected individuals show a favorable response to acetylcholinesterase (AChE) inhibitors.

    Similarity:
    Contains 1 glutamine amidotransferase type-2 domain.
    Contains 2 SIS domains.

    Database links:
    UniProtKB/Swiss-Prot: Q06210.3

    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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