Human Glucose-6-phosphatase ELISA Kit | G-6-Pase elisa kit
Human Glucose-6-phosphatase (G-6-Pase) ELISA Kit
Principle of the Assay: This assay employs a two-site sandwich ELISA to quantitate G-6-Pase in Human serum, plasma. An antibody specific for G-6-Pase has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any G-6-Pase present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for G-6-Pase is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of G-6-Pase bound in the initial step. The color development is stopped and the intensity of the color is measured.
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
G6PC: 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. Defects in G6PC are the cause of glycogen storage disease type 1A (GSD1A). 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. Belongs to the glucose-6-phosphatase family.
Protein type: Carbohydrate Metabolism - galactose; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - starch and sucrose; EC 3.1.3.9; Endoplasmic reticulum; Membrane protein, integral; Membrane protein, multi-pass; Phosphatase (non-protein); Transporter; Transporter, SLC family
Chromosomal Location of Human Ortholog: 17q21.31
Cellular Component: endoplasmic reticulum membrane; integral component of endoplasmic reticulum membrane; integral component of membrane
Molecular Function: glucose-6-phosphatase activity; phosphate binding; phosphotransferase activity, alcohol group as acceptor
Biological Process: cholesterol homeostasis; gluconeogenesis; glucose 6-phosphate metabolic process; glucose homeostasis; glucose-6-phosphate transport; glycogen catabolic process; glycogen metabolic process; multicellular organism growth; phosphorylated carbohydrate dephosphorylation; regulation of gene expression; response to food; steroid metabolic process; triacylglycerol metabolic process; urate metabolic process
Disease: Glycogen Storage Disease Ia
Research Articles on G-6-Pase
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Product Notes
The Human G-6-Pase g6pc (Catalog #AAA281778) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA281778 ELISA Kit recognizes Human G-6-Pase. It is sometimes possible for the material contained within the vial of "Glucose-6-phosphatase, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
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