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葡萄糖6磷酸酶α/G6Pase-α抗体说明书

发布时间：2025/4/18 10:41:02 阅读次数：17

产品编号：JW-4044R

英文名称：G6PC Rabbit pAb

中文名称：葡萄糖6磷酸酶α/G6Pase-α抗体

英文别名：glucose-6-phosphatase, catalytic subunit; GSD1; AW107337; G-6-Pase; G6Pase; G6Pase-alpha; Glucose 6 phosphatase alpha; G6PC_HUMAN; G6PT; Glucose-6-phosphatase alpha; Glucose-6-phosphatase; GSD1a; MGC163350; MGC93613; RP23-281C18.19.

产品应用：WB, IHC-P, Flow-Cyt, ELISA

交叉反应：Human, Rat (predicted: Mouse, Rabbit, Pig, Sheep, Cow, Dog)

抗体来源：Rabbit

免疫原：KLH conjugated synthetic peptide derived from human Glucose 6 phosphatase alpha: 81-180/357

亚型：IgG

性状：Liquid

纯化方法：affinity purified by Protein A

克隆类型：Polyclonal

理论分子量：39 kDa

浓度：1mg/ml

储存液：0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

亚细胞定位：Endoplasmic reticulum membrane; Multi-pass membrane protein.

疾病：Defects in G6PC are the cause of glycogen storage disease type 1A (GSD1A) [MIM:232200]. A metabolic disorder characterized by impairment of terminal steps of glycogenolysis and gluconeogenesis. Patients manifest a wide range of clinical symptoms and biochemical abnormalities, including hypoglycemia, severe hepatomegaly due to excessive accumulation of glycogen, kidney enlargement, growth retardation, lactic acidemia, hyperlipidemia, and hyperuricemia.

相似性：Belongs to the glucose-6-phosphatase family.

SWISS：P35575

Gene ID：2538

功能：Hydrolyzes glucose-6-phosphate to glucose in the endoplasmic reticulum. Forms with the glucose-6-phosphate transporter (SLC37A4/G6PT) the complex responsible for glucose production through glycogenolysis and gluconeogenesis. Hence, it is the key enzyme in homeostatic regulation of blood glucose levels.

保存条件：Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.