Please use this identifier to cite or link to this item: http://repositorio.unicamp.br/jspui/handle/REPOSIP/338570
Type: Artigo
Title: Disturbance of mitochondrial functions associated with permeability transition pore opening induced by cis-5-tetradecenoic and myristic acids in liver of adolescent rats
Author: Cecatto, Cristiane
Amaral, Alexandre Umpierrez
Wajner, Alessandro
Wajner, Simone Magagnin
Castilho, Roger Frigerio
Wajner, Moacir
Abstract: Patients affected by very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency commonly present liver dysfunction whose pathogenesis is poorly known. We demonstrate here that major metabolites accumulating in this disorder, namely cis-5-tetradecenoic acid (Cis-5) and myristic acid (Myr), markedly impair mitochondrial respiration, decreasing ATP production in liver mitochondrial preparations from adolescent rats. Other parameters of mitochondrial homeostasis such as membrane potential (Delta Psi m) and Ca2+ retention capacity were strongly compromised by these fatty acids, involving induction of mitochondrial permeability transition. The present data indicate that disruption of mitochondrial bioenergetics and Ca2+ homeostasis may contribute to the liver dysfunction of VLCAD deficient patients
Subject: Mitocôndrias hepáticas
Country: Reino Unido
Editor: Elsevier
Rights: Fechado
Identifier DOI: 10.1016/j.mito.2019.09.008
Address: https://www-sciencedirect.ez88.periodicos.capes.gov.br/science/article/pii/S1567724919301217
Date Issue: 2020
Appears in Collections:FCM - Artigos e Outros Documentos

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