Disturbance of mitochondrial functions associated with permeability transition pore opening induced by cis-5-tetradecenoic and myristic acids in liver of adolescent rats

Autor(es)

Cecatto, Cristiane ^[1] ; Amaral, Alexandre Umpierrez ^{[1, 2, 3]} ; Wajner, Alessandro ^[1] ; Wajner, Simone Magagnin ^[4] ; Castilho, Roger Frigerio ^[5] ; Wajner, Moacir ^{[6, 1, 2]}

Afiliação do(s) autor(es)

^[1] Univ Fed Rio Grande do Sul, Inst Ciencias Basicas Saude, Programa Posgrad Ciencias Biol Bioquim, Porto Alegre, RS - Brazil

^[2] Univ Fed Rio Grande do Sul, Inst Ciencias Basicas Saude, Dept Bioquim, Rua Ramiro Barcelos 2600 Anexo, BR-90035003 Porto Alegre, RS - Brazil

^[3] Univ Reg Integrada Alto Uruguai & Missoes, Dept Ciencias Biol, Erechim, RS - Brazil

^[4] Univ Fed Rio Grande do Sul, Fac Med, Dept Med Interna, Porto Alegre, RS - Brazil

^[5] Univ Estadual Campinas, Fac Ciencias Med, Dept Patol Clin, Campinas, SP - Brazil

^[6] Hosp Clin Porto Alegre, Serv Genet Med, Porto Alegre, RS - Brazil

• Tipo de documento: Artigo Científico
• Fonte: MITOCHONDRION; v. 50, p. 1-13, JAN 2020.
• Citações Web of Science: 0

Resumo

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. (AU)

• Processo FAPESP: 17/17728-8 - Função e disfunção mitocondrial: implicações para o envelhecimento e doenças associadas
• Beneficiário: Aníbal Eugênio Vercesi
• Modalidade de apoio: Auxílio à Pesquisa - Temático