Gene delivery corrects N-acetylglutamate synthase deficiency and enables insights in the physiological impact of L-arginine activation of N-acetylglutamate synthase

Abstract The urea cycle protects the central nervous system from ammonia toxicity by converting ammonia to urea. N-acetylglutamate synthase (NAGS) catalyzes formation of N-acetylglutamate, an essential allosteric activator of carbamylphosphate synthetase 1. Enzymatic activity of mammalian NAGS doubl...

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Bibliographic Details
Main Authors: P. Sonaimuthu, E. Senkevitch, N. Haskins, P. Uapinyoying, M. McNutt, H. Morizono, M. Tuchman, L. Caldovic
Format: Article
Language:English
Published: Nature Publishing Group 2021-02-01
Series:Scientific Reports
Online Access:https://doi.org/10.1038/s41598-021-82994-8