产品货号 :  mlR4044

英文名称 :  Glucose 6 phosphatase alpha

中文名称 :  葡萄糖6磷酸酶α/G6Pase-α抗体

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; RP23-281C18.19.  

研究领域 :  肿瘤  免疫学  转录调节因子  激酶和磷酸酶  

抗体来源 :  Rabbit

克隆类型 :  Polyclonal

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

产品应用 :  ELISA=1:500-1000 Flow-Cyt=0.2ug/test  

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user.

分 子 量 :  39kDa

细胞定位 :  细胞浆 细胞膜

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human Glucose 6 phosphatase alpha:81-180/357

亚    型 :  IgG

纯化方法 :  affinity purified by Protein A

储 存 液 :  0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

保存条件 :  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed :  PubMed

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. 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 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.

SWISS:

P35575

Gene ID:

2538

Important Note:

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

产品图片