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

AbstractPhenylketonuria (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 a nd 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 phosphotransfe...
Source: Metabolic Brain Disease - Category: Neurology Source Type: research

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Source: Biomed Res - Category: Research Authors: Tags: Biomed Res Int Source Type: research
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Source: Biomed Res - Category: Research Authors: Tags: Biomed Res Int Source Type: research
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Source: Biomed Res - Category: Research Authors: Tags: Biomed Res Int Source Type: research
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Source: Biomed Res - Category: Research Authors: Tags: Biomed Res Int Source Type: research
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Source: Neuroscience - Category: Neuroscience Source Type: research
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Source: Neural Plasticity - Category: Neurology Authors: Tags: Neural Plast Source Type: research
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Source: Behavioural Neurology - Category: Neurology Authors: Tags: Behav Neurol Source Type: research
Phenylketonuria (PKU) is the most frequent hereditary metabolic disorder in our environment. Its frequency ranges between 1 / 4000-40,000 live births. PKU is an autosomal recessive inborn error of phenylalanine (phe) metabolism caused by deficiency in the enzyme phenylalanine hydroxylase that converts phenylalanine, together with its cofactor tetrahydrobiopterin (BH4), into tyrosine. If left untreated, PKU results in increased phenylalanine concentrations in blood and brain, which cause severe intellectual disability, epilepsy and behavioural problems.
Source: Nutrition - Category: Nutrition Authors: Source Type: research
Conclusion Reports on the clinical and electrophysiological features of adult patients with PKU were scant. We emphasized that reflex clinical features may be observed in this metabolic disease, and focal epileptiform abnormalities and asymmetry may be present in electrophysiological evaluation besides the rare association with HS.
Source: Epilepsy and Behavior - Category: Neurology Source Type: research
CONCLUSION: Reports on the clinical and electrophysiological features of adult patients with PKU were scant. We emphasized that reflex clinical features may be observed in this metabolic disease, and focal epileptiform abnormalities and asymmetry may be present in electrophysiological evaluation besides the rare association with HS. PMID: 29579554 [PubMed - as supplied by publisher]
Source: Epilepsy and Behaviour - Category: Neurology Authors: Tags: Epilepsy Behav Source Type: research
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