Difference between revisions of "Cecatto 2020 Mitochondrion"
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{{Publication | {{Publication | ||
|title=Cecatto C, Amaral AU, Wajner A, Wajner SM, Castilho RF, Wajner M ( | |title=Cecatto C, Amaral AU, Wajner A, Wajner SM, Castilho RF, Wajner M (2020) 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. | ||
|info=[https://www.ncbi.nlm.nih.gov/pubmed/31655165 PMID: 31655165 Open Access] | |info=[https://www.ncbi.nlm.nih.gov/pubmed/31655165 PMID: 31655165 Open Access] | ||
|authors=Cecatto C, Amaral AU, Wajner A, Wajner SM, Castilho RF, Wajner M | |authors=Cecatto C, Amaral AU, Wajner A, Wajner SM, Castilho RF, Wajner M | ||
|year= | |year=2020 | ||
|journal=Mitochondrion | |journal=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 (ΔΨm) and Ca<sup>2+</sup>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 Ca<sup>2+</sup>homeostasis may contribute to the liver dysfunction of VLCAD deficient patients. | |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 Ca<sup>2+</sup>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 Ca<sup>2+</sup>homeostasis may contribute to the liver dysfunction of VLCAD deficient patients. | ||
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<small>Copyright © 2019 Elsevier B.V. and Mitochondria Research Society. All rights reserved.</small> | <small>Copyright © 2019 Elsevier B.V. and Mitochondria Research Society. All rights reserved.</small> | ||
|keywords=Cis-5-tetradecenoic acid, Liver mitochondria, Mitochondrial homeostasis, Mitochondrial permeability transition, Myristic acid, VLCAD deficiency | |keywords=Cis-5-tetradecenoic acid, Liver mitochondria, Mitochondrial homeostasis, Mitochondrial permeability transition, Myristic acid, VLCAD deficiency | ||
|editor=[[Plangger M]] | |editor=[[Plangger M]] | ||
|mipnetlab=BR Porto Alegre Souza DOG | |mipnetlab=BR Porto Alegre Souza DOG | ||
}} | }} | ||
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|pathways=N, S | |pathways=N, S | ||
|instruments=Oxygraph-2k | |instruments=Oxygraph-2k | ||
|additional=Labels, 2019-11 | |additional=Labels, 2019-11 | ||
}} | }} | ||
Revision as of 15:39, 22 September 2021
| Cecatto C, Amaral AU, Wajner A, Wajner SM, Castilho RF, Wajner M (2020) 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 (2020) 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 (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
Labels, 2019-11
