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GFPT1 Rabbit pAb (bs-13341R)  
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產(chǎn)品編號 bs-13341R
英文名稱 GFPT1 Rabbit pAb
中文名稱 谷氨酰胺6-磷酸果糖轉(zhuǎn)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(xì)胞生物  信號轉(zhuǎn)導(dǎo)  糖尿病  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human (predicted: Mouse,Rat,Rabbit,Pig,Sheep,Chicken,Dog,Horse)
產(chǎn)品應(yīng)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(xì)胞定位 細(xì)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(xiàng) 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 goa
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