Glutamate is a central junction for interchange of amino nitrogen, glutamate facilitates both amino acid synthesis and degradation.

 In the liver, glutamate is the terminus for release of ammonia from amino acids, and the intrahepatic concentration of glutamate modulates the rate of ammonia detoxification into urea.
 In pancreatic beta-cells, oxidation of glutamate mediates amino acid-stimulated insulin secretion.
 In the central nervous system, glutamate serves as an excitatory neurotransmittor. Glutamate is also the precursor of the inhibitory neurotransmittor GABA, as well as glutamine, a potential mediator of hyperammonemic neurotoxicity.

Pathology

 muttaions of the glutamate dehydrogenase in a form of congenital hyperinsulinism associated with asymptomatic hyperammonemia

 disruptions of glutamate metabolism

• pyridoxine-dependent seizures

Features

 glutamate oxidation

References

 - Kelly A, Li C, Gao Z, Stanley CA, Matschinsky FM. Glutaminolysis and insulin secretion: from bedside to bench and back. Diabetes. 2002 Dec;51 Suppl 3:S421-6. PMID: #12475785#

 Kelly A, Stanley CA. Disorders of glutamate metabolism. Ment Retard Dev Disabil Res Rev. 2001;7(4):287-95. PMID: #11754524#