Cecatto 2019 Mitochondrion
|Cecatto C, Amaral AU, Wajner A, Wajner SM, Castilho RF, Wajner M (2019) Disturbance of mitochondrial functions associated with permeability transition pore opening induced by cis-5-tetradecenoic and myristic acids in liver of adolescent rats. Mitochondrion 50:1-13.|
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 (ΔΨm) 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.
Copyright © 2019 Elsevier B.V. and Mitochondria Research Society. All rights reserved.
• Keywords: Cis-5-tetradecenoic acid, Liver mitochondria, Mitochondrial homeostasis, Mitochondrial permeability transition, Myristic acid, VLCAD deficiency • Bioblast editor: Plangger M • O2k-Network Lab: BR Porto Alegre Souza DOG
Labels: MiParea: Respiration, Pharmacology;toxicology Pathology: Inherited, Other Stress:Permeability transition Organism: Rat Tissue;cell: Liver, Other cell lines Preparation: Permeabilized cells, Isolated mitochondria
Regulation: Calcium, mt-Membrane potential, Fatty acid Coupling state: LEAK, OXPHOS, ET Pathway: N, S HRR: Oxygraph-2k