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 GSand 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 GSin 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: Energy Metabolism - nitrogen; Ligase; EC 4.1.1.15; Amino Acid Metabolism - arginine and proline; Amino Acid Metabolism - alanine, aspartate and glutamate; EC 6.3.1.2
Chromosomal Location of Human Ortholog: 1q31
Cellular Component: cytoplasm; cytosol; mitochondrion; myelin sheath; nerve terminal; nucleus; perikaryon; protein complex; rough endoplasmic reticulum
Molecular Function: ATP binding; dynein light chain binding; glutamate binding; glutamate decarboxylase activity; glutamate-ammonia ligase activity; identical protein binding; magnesium ion binding; manganese ion binding
Biological Process: amino acid biosynthetic process; cell proliferation; cellular response to starvation; glutamate catabolic process; glutamine biosynthetic process; neurotransmitter uptake; positive regulation of epithelial cell proliferation; positive regulation of insulin secretion; positive regulation of synaptic transmission, glutamatergic; protein homooligomerization; response to glucose stimulus; synaptic transmission
Disease: Glutamine Deficiency, Congenital