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G6PC Rabbit pAb
- 产品详情
- 实验流程
Application 
| WB, IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | P35575 |
| Other Accession | P35575 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 40484 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human Glucose 6 phosphatase alpha |
| Epitope Specificity | 81-180/357 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the glucose-6-phosphatase family. |
| 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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] |
| Gene ID | 2538 |
|---|---|
| Other Names | G6PC; G6Pase; G6PT; GSD1; GSD1a; Glc-6-Pase; Psme3; G6PC1_CANLF; G6PC1; Glucose-6-phosphatase (G-6-Pase | G6Pase); 3.1.3.9; G6PC1_HUMAN; Glucose-6-phosphatase alpha (G6Pase-alpha); G6PC1_MOUSE; G6PC1_RAT; |
| Dilution | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,Flow-Cyt=0.2ug/test |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. 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. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | G6PC1 (HGNC:4056) |
|---|---|
| Synonyms | G6PC, G6PT |
| 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 in the terminal step of glycogenolysis and gluconeogenesis. Hence, it is the key enzyme in homeostatic regulation of blood glucose levels. |
| Cellular Location | Endoplasmic reticulum membrane; Multi-pass membrane protein |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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¥ 1,250.00
Cat# AP99839
