特記事項（精製）This product is an IgG fraction antibody purified from monospecific antiserum by a multistep process which includes delipidation, salt fractionation and ion exchange chromatography followed by extensive dialysis against the buffer stated above.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
追加情報Dot: Use at an assay dependent dilution.
ELISA: 1/4000 - 1/20000. This product has been assayed against 1.0?g of Glutamine Synthetase in a standard capture ELISA using Peroxidase Conjugated Streptavidin and ABTS as a substrate for 30 minutes at room temperature.
ICC/IF: Use at an assay dependent dilution.
WB: Use at an assay dependent dilution. Predicted molecular weight: 42 kDa.
Suitable for antibody based assays using streptavidin or avidin conjugates requiring lot-to-lot consistency.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
機能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 (By similarity). Essential for proliferation of fetal skin fibroblasts.
関連疾患Defects in GLUL are the cause of congenital systemic glutamine deficiency (CSGD) [MIM:610015]. 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.