Protein requirements in adults with phenylketonuria and bioavailability of glycomacropeptide compared to an l-amino acid-based product.

ABSTRACT:

Background

Phenylketonuria (PKU) is caused by phenylalanine hydroxylase deficiency. Treatment is primarily a low-Phe diet combined with l-amino acid-based products (l-AA). Protein requirements in adults with PKU have not been directly determined. A formula with glycomacropeptide (GMP) and low phenylalanine is available, yet untested for optimal protein synthesis.

Objectives

To determine the protein requirements in adults with PKU and the bioavailability of GMP-AA in the same patients using the indicator amino acid oxidation (IAAO) technique.

Methods

Each participant was allocated to 7 separate l-AA intakes (range: 0.1-1.8 g/kg/day) in Experiment 1. In Experiment 2, the same patients participated in 4 GMP-AA intakes (range: 0.1-0.9 g/kg/day). The IAAO method with l-[1-

SUBMITTER: Turki A 

PROVIDER: S-EPMC11670041 | biostudies-literature | 2025 Jan

REPOSITORIES: biostudies-literature