Large neutral amino acids in the treatment of PKU
| Autor: | Martijn de Groot, Margreet van Rijn, Marieke Hoeksma, Francjan J. van Spronsen, Dirk-Jan Reijngoud |
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| Přispěvatelé: | Groningen University Institute for Drug Exploration (GUIDE), Center for Liver, Digestive and Metabolic Diseases (CLDM) |
| Jazyk: | angličtina |
| Rok vydání: | 2010 |
| Předmět: |
medicine.medical_specialty
Phenylketonurias LEUCINE Advances and Challenges in PKU Phenylalanine Theory to practice Biology Reference Daily Intake chemistry.chemical_compound CEREBROSPINAL-FLUID Internal medicine Genetics medicine Animals Humans Genetics(clinical) Neurotransmitter PHENYLKETONURIA Genetics (clinical) Essential amino acid chemistry.chemical_classification Food Formulated Rationalization PHENYLALANINE RESTRICTION ISOLEUCINE BLOOD-BRAIN-BARRIER GENETIC MOUSE MODEL SEROTONIN Professional Practice Models Theoretical TRANSPORT Amino acid Endocrinology Amino Acids Neutral chemistry TYROSINE SUPPLEMENTATION Dietary Supplements Leucine |
| Zdroj: | Journal of Inherited Metabolic Disease Journal of Inherited Metabolic Disease, 33(6), 671-676. SPRINGER |
| ISSN: | 0141-8955 |
| Popis: | Notwithstanding the success of the traditional dietary phenylalanine restriction treatment in phenylketonuria (PKU), the use of large neutral amino acid (LNAA) supplementation rather than phenylalanine restriction has been suggested. This treatment modality deserves attention as it might improve cognitive outcome and quality of life in patients with PKU. Following various theories about the pathogenesis of cognitive dysfunction in PKU, LNAA supplementation may have multiple treatment targets: a specific reduction in brain phenylalanine concentrations, a reduction in blood (and consequently brain) phenylalanine concentrations, an increase in brain neurotransmitter concentrations, and an increase in brain essential amino acid concentrations. These treatment targets imply different treatment regimes. This review summarizes the treatment targets and the treatment regimens of LNAA supplementation and discusses the differences in LNAA intake between the classical dietary phenylalanine-restricted diet and several LNAA treatment forms. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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