Secondary BH4 deficiency links protein homeostasis to regulation of phenylalanine metabolism
Abstract
Metabolic control of phenylalanine concentrations in body fluids is essential for cognitive development and executive function. The hepatic phenylalanine hydroxylating system is regulated by the ratio of l-phenylalanine, which is substrate of phenylalanine hydroxylase (PAH), to the PAH cofactor tetrahydrobiopterin (BH4). Physiologically, phenylalanine availability is governed by nutrient intake, whereas liver BH4 is kept at constant level. In...
Paper Details
Title
Secondary BH4 deficiency links protein homeostasis to regulation of phenylalanine metabolism
Published Date
Mar 5, 2018
Journal
Volume
27
Issue
10
Pages
1732 - 1742
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