Principle of the Assay: This ELISA kit uses Sandwich-ELISA as the method. The micro ELISA plate provided in this kit has been pre-coated with an antibody specific to GS. Standards or samples are added to the appropriate micro ELISA plate wells and combined with the specific antibody. Then a biotinylated detection antibody specific for GS and Avidin-Horseradish Peroxidase (HRP) conjugate is added to each micro plate well successively and incubated. Free components are washed away. The substrate solution is added to each well. Only those wells that contain GS, biotinylated detection antibody and Avidin-HRP conjugate will appear blue in color. The enzyme-substrate reaction is terminated by the addition of a sulphuric acid solution and the color turns yellow. The optical density (OD) is measured spectrophotometrically at a wavelength of 450 nm +/- 2 nm. The OD value is proportional to the concentration of GS. You can calculate the concentration of GS in the samples by comparing the OD of the samples to the standard curve.
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene belongs to the glutamine synthetase family. It catalyzes the synthesis of glutamine from glutamate and ammonia in an ATP-dependent reaction. This protein plays a role in ammonia and glutamate detoxification, acid-base homeostasis, cell signaling, and cell proliferation. Glutamine is an abundant amino acid, and is important to the biosynthesis of several amino acids, pyrimidines, and purines. Mutations in this gene are associated with congenital glutamine deficiency, and overexpression of this gene was observed in some primary liver cancer samples. There are six pseudogenes of this gene found on chromosomes 2, 5, 9, 11, and 12. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2014]
Uniprot Description
GLUL: This enzyme has 2 functions: it catalyzes the production of glutamine and 4-aminobutanoate (gamma-aminobutyric acid, GABA), the latter in a pyridoxal phosphate-independent manner. Essential for proliferation of fetal skin fibroblasts. Defects in GLUL are the cause of congenital systemic glutamine deficiency (CSGD). CSGD is a rare developmental disorder with severe brain malformation resulting in multi-organ failure and neonatal death. Glutamine is largely absent from affected patients serum, urine and cerebrospinal fluid. Belongs to the glutamine synthetase family.
Protein type: Amino Acid Metabolism - alanine, aspartate and glutamate; EC 4.1.1.15; Amino Acid Metabolism - arginine and proline; Ligase; Energy Metabolism - nitrogen; EC 6.3.1.2
Chromosomal Location of Human Ortholog: 1q31
Cellular Component: protein complex; mitochondrion; rough endoplasmic reticulum; cytoplasm; perikaryon; nerve terminal; nucleus; cytosol
Molecular Function: glutamate-ammonia ligase activity; identical protein binding; glutamate binding; dynein light chain binding; glutamate decarboxylase activity; manganese ion binding; magnesium ion binding; ATP binding
Biological Process: cell proliferation; glutamate catabolic process; synaptic transmission; glutamine biosynthetic process; response to glucose stimulus; positive regulation of insulin secretion; neurotransmitter uptake; amino acid biosynthetic process; cellular response to starvation; positive regulation of synaptic transmission, glutamatergic; protein homooligomerization; positive regulation of epithelial cell proliferation
Disease: Glutamine Deficiency, Congenital
Research Articles on GS
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Product Notes
The Guinea Pig GS glul (Catalog #AAA2500246) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA2500246 ELISA Kit recognizes Guinea Pig GS. It is sometimes possible for the material contained within the vial of "GS, 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
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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