葡萄糖6磷酸酶α/G6Pase-α抗体
规格：1mg/1ml
英文名： Glucose 6 phosphatase alpha
别名： glucose-6-phosphatase, catalytic subunit; GSD1; AW107337; G-6-Pase; G6Pase; G6Pase-alpha; g6pc; G6PC_HUMAN; G6PT; Glucose-6-phosphatase alpha; Glucose-6-phosphatase; GSD1a; MGC163350; MGC93613; RP23-2
分子量： 39kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Glucose
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, Sheep,
细胞定位：细胞浆 细胞膜
葡萄糖6磷酸酶α/G6Pase-α抗体产品介绍：background: Glucose-6-phosphatase (G6Pase) is a multi-subunit integral membrane protein of the endoplasmic reticulum that is composed of a catalytic subunit and transporters for G6P, inorganic phosphate, and glucose. This gene (G6PC) is one of the three glucose-6-phosphatase catalytic-subunit-encoding genes in human: G6PC, G6PC2 and G6PC3. Glucose-6-phosphatase catalyzes the hydrolysis of D-glucose 6-phosphate to D-glucose and orthophosphate and is a key enzyme in glucose homeostasis, functioning in gluconeogenesis and glycogenolysis. Mutations in this gene cause glycogen storage disease type I (GSD1). This disease, also known as von Gierke disease, is a metabolic disorder characterized by severe hypoglycemia associated with the accumulation of glycogen and fat in the liver and kidneys.[provided by RefSeq, Feb 2011] Function: 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. 葡萄糖6磷酸酶α/G6Pase-α抗体Hence, it is the key enzyme in homeostatic regulation of blood glucose levels. Subcellular Location: Endoplasmic reticulum membrane; Multi-pass membrane protein. DISEASE: 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 葡萄糖6磷酸酶α/G6Pase-α抗体symptoms and biochemical abnormalities, including hypoglycemia, severe hepatomegaly due to excessive accumulation of glycogen, kidney enlargement, growth retardation, lactic acidemia, hyperlipidemia, and hyperuricemia. Similarity: Belongs to the glucose-6-phosphatase family. Gene ID: 2538 Database links: Entrez Gene: 403492 Dog Entrez Gene: 2538 Human Entrez Gene: 14377 Mouse Entrez Gene: 25634 Rat SwissProt: O19133 Dog SwissProt: P35575 Human SwissProt: P35576 Mouse SwissProt: P43428 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
葡萄糖6磷酸酶α/G6Pase-α抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 **学 转录调节因子 激酶和磷酸酶
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid