Preprint Review Version 1 Preserved in Portico This version is not peer-reviewed

Supplementation for Performance and Health in Patients with Phenylketonuria: An Exercise-Based Approach to Improving Dietary Adherence

Version 1 : Received: 4 February 2024 / Approved: 6 February 2024 / Online: 6 February 2024 (08:38:06 CET)

A peer-reviewed article of this Preprint also exists.

González-Lamuño, D.; Morencos, C.; Arrieta, F.J.; Venegas, E.; Vicente-Rodríguez, G.; Casajús, J.A.; Couce, M.L.; Aldámiz-Echevarría, L. Supplementation for Performance and Health in Patients with Phenylketonuria: An Exercise-Based Approach to Improving Dietary Adherence. Nutrients 2024, 16, 639. González-Lamuño, D.; Morencos, C.; Arrieta, F.J.; Venegas, E.; Vicente-Rodríguez, G.; Casajús, J.A.; Couce, M.L.; Aldámiz-Echevarría, L. Supplementation for Performance and Health in Patients with Phenylketonuria: An Exercise-Based Approach to Improving Dietary Adherence. Nutrients 2024, 16, 639.

Abstract

Proteins play a pivotal role in supporting athletes by promoting muscle hypertrophy and minimizing protein catabolism during exercise. They stimulate muscle protein synthesis, aiding in the repair of exercise-induced muscle damage and serving as an energy source, particularly for post-exercise glycogen replenishment. In individuals with phenylketonuria (PKU), for whom protein intake is limited to essential phenylalanine (Phe), supplementation with Phe-free amino acids or glycomacropeptides is necessary. Tailored for macronutrient and micronutrient content, these protein substitutes fulfill most protein requirements, but lack Phe, unlike dietary sources of protein. Protein requirements for athletes with PKU exceed general recommendations due to limited availability in supplements and potential catabolism mitigation. Various factors influence tolerated dietary Phe in PKU, including protein substitute quantity, distribution, pharmacological treatment, age, growth rate, pregnancy, catabolic states, and physical activity. While general protein requirements for PKU surpass those of the general population, adherence to supplement regimens is crucial for athletes and patients with PKU. Strategically timed protein substitutes enhance muscle protein synthesis, body composition, and sports performance within safety limits. Individualized intake is essential for optimal outcomes, as proteins have a multifaceted role in optimizing athletic performance and addressing challenges in PKU.

Keywords

Phenylketonuria; protein substitutes; amino acid; glycomacropetides; phenyalanine; tyrosine; physical activity; supplement regimens; adherence

Subject

Public Health and Healthcare, Physical Therapy, Sports Therapy and Rehabilitation

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