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Creatine plus pyruvate supplementation prevents oxidative stress and phosphotransfer network disturbances in the brain of rats subjected to chemically-induced phenylketonuria.

Abstract：

:Phenylketonuria (PKU) is the most common inborn error of amino acid metabolism. Usually diagnosed within the first month of birth, it is essential that the patient strictly follow the dietary restriction of natural protein intake. Otherwise, PKU impacts the development of the brain severely and may result in microcephaly, epilepsy, motor deficits, intellectual disability, and psychiatric and behavioral disorders. The neuropathology associated with PKU includes defects of myelination, insufficient synthesis of monoamine neurotransmitters, amino acid imbalance across the blood-brain barrier, and involves intermediary metabolic pathways supporting energy homeostasis and antioxidant defenses in the brain. Considering that the production of reactive oxygen species (ROS) is inherent to energy metabolism, we investigated the association of creatine+pyruvate (Cr + Pyr), both energy substrates with antioxidants properties, as a possible treatment to mitigate oxidative stress and phosphotransfer network impairment elicited in the brain of young Wistar rats by chemically-induced PKU. We induced PKU through the administration of α-methyl-L-phenylalanine and phenylalanine for 7 days, with and without Cr + Pyr supplementation, until postpartum day 14. The cotreatment with Cr + Pyr administered concurrently with PKU induction prevented ROS formation and part of the alterations observed in antioxidants defenses and phosphotransfer network enzymes in the cerebral cortex, hippocampus, and cerebellum. If such prevention also occurs in PKU patients, supplementing the phenylalanine-restricted diet with antioxidants and energetic substrates might be beneficial to these patients.

journal_name

Metab Brain Dis

journal_title

Metabolic brain disease

authors

Bortoluzzi VT,Brust L,Preissler T,de Franceschi ID,Wannmacher CMD

doi

10.1007/s11011-019-00472-7

subject

Has Abstract

pub_date

2019-12-01 00:00:00

pages

1649-1660

issue

eissn

0885-7490

issn

1573-7365

pii

10.1007/s11011-019-00472-7

journal_volume

pub_type

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Creatine plus pyruvate supplementation prevents oxidative stress and phosphotransfer network disturbances in the brain of rats subjected to chemically-induced phenylketonuria.