蛋白名称
Glutamine synthetase
特异性
This antibody detects endogenous levels of Gl Syn/Glutamine Synthetase protein.
组成
PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
来源
Mouse Monoclonal antibody
稀释比例
IHC 1:200-1000;WB 1:500-2000;IF 1:100-500;ELISA 1:1000-5000;
纯化工艺
The antibody was affinity-purified from ascites by affinity-chromatography using specific immunogen.
其他名称
GLUL ;
GLNS ;
Glutamine synthetase ;
GS ;
Glutamate decarboxylase ;
Glutamate--ammonia ligase
背景
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],
功能
Catalytic activity:ATP + L-glutamate + NH(3) = ADP + phosphate + L-glutamine.,Disease: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.,online information:Glutamine synthetase entry,similarity:Belongs to the glutamine synthetase family.,subunit:Homooctamer.,
