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    GFPT1 Rabbit pAb (bs-13341R)  
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    產(chǎn)品編號 bs-13341R
    英文名稱(chēng) GFPT1 Rabbit pAb
    中文名稱(chēng) 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.  
    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
    克 隆 號
    交叉反應 Human (predicted: Mouse,Rat,Rabbit,Pig,Sheep,Chicken,Dog,Horse)
    產(chǎn)品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 79 kDa
    檢測分子量
    細胞定位 細胞漿 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human GFPT1: 601-699/699 
    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 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.

    SWISS:
    Q06210

    Gene ID:
    2673

    Database links:

    Entrez Gene: 2673 Human

    Entrez Gene: 14583 Mouse

    Entrez Gene: 297417 Rat

    Omim: 138292 Human

    SwissProt: Q06210 Human

    SwissProt: P47856 Mouse

    SwissProt: P82808 Rat

    Unigene: 580300 Human

    Unigene: 19893 Mouse

    Unigene: 229622 Rat



    產(chǎn)品圖片
    Tissue/cell: human lung carcinoma; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-GFPT1 Polyclonal Antibody, Unconjugated(bs-13341R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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