| Literature DB >> 31410688 |
Nathana Jamille Mezzomo1, Diego Becker Borin2, Francine Ianiski2, Barbara Dotto Fontana2, Itiane Diehl de Franceschi3, Juliane Bolzan2, Renata Garcez2, Mateus Grings3, Belisa Parmeggiani3, Liana da Silva Fernandes2, Rodrigo de Almeida Vaucher2, Guilhian Leipnitz3, Clovis Milton Duval Wannmacher3, Virginia Cielo Rech4.
Abstract
Phenylketonuria (PKU) is a metabolic disorder accumulating phenylalanine (Phe) and its metabolites in plasma and tissues of the patients. Regardless of the mechanisms, which Phe causes brain impairment, are poorly understood, energy deficit may have linked to the neurotoxicity in PKU. It is widely recognized that creatine is involved in maintaining of cerebral energy homeostasis. Because of this, in a previous work, we incorporated it into liposomes and this increased the concentration of creatine in the cerebral cortex. Here, we examined the effect of creatine nanoliposomes on parameters of oxidative stress, enzymes of phosphoryl transfer network, and activities of the mitochondrial respiratory chain complexes (RCC) in the cerebral cortex of young rats chemically induced hyperphenylalaninemia (HPA). HPA was induced with L-phenylalanine (5.2 µmol/g body weight; twice a day; s.c.), and phenylalanine hydroxylase inhibitor, α-methylphenylalanine (2.4 µmol/g body weight; once a day; i.p.), from the 7th to the 19th day of life. HPA reduced the activities of pyruvate kinase, creatine kinase, and complex II + III of RCC in the cerebral cortex. Creatine nanoliposomes prevented the inhibition of the activities of the complexes II + III, caused by HPA, and changes oxidative profile in the cerebral cortex. Considering the importance of the mitochondrial respiratory chain for brain energy production, our results suggesting that these nanoparticles protect against neurotoxicity caused by HPA, and can be viable candidates for treating patients HPA.Entities:
Keywords: Brain energy metabolism; Hyperphenylalaninemia; Nanoparticle; Neurotoxicity; Phenylketonuria; Respiratory chain complexes
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Year: 2019 PMID: 31410688 DOI: 10.1007/s11033-019-05023-y
Source DB: PubMed Journal: Mol Biol Rep ISSN: 0301-4851 Impact factor: 2.316