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Rabbit Anti-GFPT1/Gold Conjugated antibody (bs-13341R-Gold)
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说 明 书: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包装/询价
产品编号 bs-13341R-Gold
英文名称 Rabbit Anti-GFPT1/Gold Conjugated antibody
中文名称 胶体金标记的谷氨酰胺6-磷酸果糖转移酶抗体
别    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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul(10nm  15nm  35nm
研究领域 细胞生物  信号转导  糖尿病  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human,  (predicted: Mouse, Rat, Chicken, Dog, Pig, Horse, Rabbit, Sheep, )
产品应用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 79kDa
性    状 Lyophilized or Liquid
浓    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GFPT1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存条件 Store at 2-8 ºC for 3-6 months. Avoid repeated freeze/thaw cycles.
产品介绍 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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