Full Text Inquiry
ePub [ahead of print] Inquiry Form:

Thank you for your interest in the full text of this ePub article. The full text of this article is not available as yet. Could you please complete and submit the brief EPub full text inquiry form given below and one of our representatives will contact you shortly with details of the article, it's availability, and price on order.


Article Detail:

Title:
Glutamate Dehydrogenase as a Promising Target for Hyperinsulinism Hyperammonemia Syndrome Therapy.

Abstract:
Hyperinsulinism-hyperammonemia syndrome (HHS) is a rare disease characterized by recurrent hypoglycemia and persistent elevation of plasma ammonia, and it can lead to severe epilepsy and permanent brain damage. It has been demonstrated that functional mutations of glutamate dehydrogenase (GDH), an enzyme in the mitochondrial matrix, are responsible for the HHS. Thus, GDH has become a promising target for the small molecule therapeutic intervention of HHS. Several medicinal chemistry studies are currently aimed at GDH, however, to date, none of the compounds reported has been entered clinical trials. This perspective summarizes the progress in the discovery and development of GDH inhibitors, including the pathogenesis of HHS, potential binding sites, screening methods, and research models. Future therapeutic perspectives are offered to provide a reference for discovering potent GDH modulators and encourage additional research that will provide more comprehensive guidance for drug development.

Journal Title: Current Medicinal Chemistry

Price: $95


Inquiry Form

6 + 0 =
Solve this simple math problem and enter the result. E.g. for 1+3, enter 4.