Cecatto 2020 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. |
Cecatto C, Amaral AU, Wajner A, Wajner SM, Castilho RF, Wajner M (2019) Mitochondrion
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 (ΞΞ¨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.
Copyright Β© 2019 Elsevier B.V. and Mitochondria Research Society. All rights reserved. β’ Keywords: Cis-5-tetradecenoic acid, Liver mitochondria, Mitochondrial homeostasis, Mitochondrial permeability transition, Myristic acid, VLCAD deficiency β’ Bioblast editor: Plangger M
Labels: MiParea: Respiration
HRR: Oxygraph-2k
Labels, 2019-11
