In Vivo Metabolic Responses to Different Formulations of Amino Acid Mixtures for the Treatment of Phenylketonuria (PKU)
| Autor: | Nadia Giarratana, Luciana Giardino, Andrea Bighinati, Giorgio Reiner, Júlio César Rocha |
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| Přispěvatelé: | NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM) |
| Jazyk: | angličtina |
| Rok vydání: | 2022 |
| Předmět: |
Male
Catalysis Metabolic control Inorganic Chemistry Prolonged release Phenylketonurias BUN Oxidation Diet Protein-Restricted Animals Amino Acids Rats Wistar Physical and Theoretical Chemistry Molecular Biology Spectroscopy Phenylketonuria (PKU) amino acid mixtures Organic Chemistry Caseins Membrane Proteins Catabolism General Medicine Myostatin Rats Computer Science Applications phenylketonuria (PKU) amino acid mixtures metabolic control prolonged release catabolism oxidation Biomarkers |
| Zdroj: | International Journal of Molecular Sciences; Volume 23; Issue 4; Pages: 2227 |
| Popis: | Funding Information: Conflicts of Interest: This study was financially supported by APR Applied Pharma Research S.A. (Balerna, Switzerland). N.G. and G.R. are employees of APR Applied Pharma Research S.A. (Balerna, Switzerland); L.C. and A.B. work at DIMIVET, an Italian contract research organization associated with Bologna University, with no financial interest in APR Applied Pharma Research S.A.; J.C.R. is a member of the European Nutritionist Expert Panel (Biomarin), the Advisory Board for Applied Pharma Research and Nutricia, and has received honoraria as a speaker from APR, Merck Serono, Biomarin, Nutricia, Vitaflo, Cambrooke, PIAM and Lifediet. Publisher Copyright: © 2022 by the authors. Licensee MDPI, Basel, Switzerland. Phenylketonuria (PKU) is a rare autosomal recessive inborn error of metabolism where the mainstay of treatment is a Phe restricted diet consisting of a combination of limited amounts of natural protein with supplementation of Phe-free or low-Phe protein substitutes and special low protein foods. Suboptimal outcomes may be related to the different absorption kinetics of free AAs, which have lower biological efficacy than natural proteins. Physiomimic Technology™ is a technology engineered to prolong AA (AA-PT) release allowing physiological absorption and masking the odor and taste of free AAs. The aim of these studies was to assess the impact of AA-PT formulation on selected functional and metabolic parameters both in acute and long-term experimental studies. Adult rats in fasting conditions were randomized in different groups and treated by oral gavage. Acute AA-PT administration resulted in significantly lower BUN at 90 min versus baseline. Both BUN and glycemia were modulated in the same direction as intact casein protein. Long-term treatment with AA-PT significantly reduces the protein expression of the muscle degradation marker Bnip3L (−46%) while significantly increasing the proliferation of market myostatin (+58%). Animals dosed for 15 days with AA-PT had significantly stronger grip strength (+30%) versus baseline. In conclusion, the results suggest that the AA-PT formulation may have beneficial effects on both AA oxidation and catabolism with a direct impact on muscle as well as on other metabolic pathways. publishersversion published |
| Databáze: | OpenAIRE |
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