Protein Substitutes in PKU; Their Historical Evolution.
Amino Acids
/ administration & dosage
Animals
Caseins
/ administration & dosage
Child, Preschool
Dietary Proteins
/ administration & dosage
Female
History, 20th Century
History, 21st Century
Humans
Nutritional Requirements
Peptide Fragments
/ administration & dosage
Phenylalanine
/ blood
Phenylketonurias
/ blood
Phenylpyruvic Acids
/ urine
Protein Hydrolysates
/ administration & dosage
United Kingdom
amino acid
glycomacropeptide
phenylketonuria
protein substitute
Journal
Nutrients
ISSN: 2072-6643
Titre abrégé: Nutrients
Pays: Switzerland
ID NLM: 101521595
Informations de publication
Date de publication:
02 Feb 2021
02 Feb 2021
Historique:
received:
29
12
2020
revised:
27
01
2021
accepted:
28
01
2021
entrez:
5
2
2021
pubmed:
6
2
2021
medline:
30
4
2021
Statut:
epublish
Résumé
Protein substitutes developed for phenylketonuria (PKU) are a synthetic source of protein commonly based on L-amino acids. They are essential in the treatment of phenylketonuria (PKU) and other amino acid disorders, allowing the antagonistic amino acid to be removed but with the safe provision of all other amino acids necessary for maintaining normal physiological function. They were first formulated by a chemist and used experimentally on a 2-year-old girl with PKU and their nutritional formulations and design have improved over time. Since 2008, a bioactive macropeptide has been used as a base for protein substitutes in PKU, with potential benefits of improved bone and gut health, nitrogen retention, and blood phenylalanine control. In 2018, animal studies showed that physiomimic technology coating the amino acids with a polymer allows a slow release of amino acids with an improved physiological profile. History has shown that in PKU, the protein substitute's efficacy is determined by its nutritional profile, amino acid composition, dose, timing, distribution, and an adequate energy intake. Protein substitutes are often given little importance, yet their pharmacological actions and clinical benefit are pivotal when managing PKU.
Identifiants
pubmed: 33540516
pii: nu13020484
doi: 10.3390/nu13020484
pmc: PMC7912909
pii:
doi:
Substances chimiques
Amino Acids
0
Caseins
0
Dietary Proteins
0
Peptide Fragments
0
Phenylpyruvic Acids
0
Protein Hydrolysates
0
caseinomacropeptide
0
Phenylalanine
47E5O17Y3R
phenylpyruvic acid
X7CO62M413
Types de publication
Historical Article
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
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