Mitochondrial bioenergetics deregulation caused by long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies in rat brain: A possible role of mPTP opening as a pathomechanism in these disorders?
- 1 September 2014
- journal article
- Published by Elsevier in Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
- Vol. 1842 (9) , 1658-1667
Abstract
No abstract availableKeywords
Funding Information
- Conselho Nacional de Desenvolvimento Científico e Tecnológico (470236/2012-4)
- Fundação de Amparo à Pesquisa do Estado do Rio Grande do Sul (10/0031-1)
- Financiadora de Estudos e Projetos (01.06.0842-00)
- Pró-Reitoria de Pesquisa, Universidade Federal do Rio Grande do Sul (PIBITI 18489)
- Instituto Nacional de Ciência e Tecnologia para Excitotoxicidade e Neuroproteção (573677/2008-5)
This publication has 79 references indexed in Scilit:
- Neuropsychological outcomes in fatty acid oxidation disorders: 85 cases detected by newborn screeningDevelopmental Disabilities Research Reviews, 2013
- Genetic and cellular modifiers of oxidative stress: What can we learn from fatty acid oxidation defects?Molecular Genetics and Metabolism, 2013
- Long-chain 3-hydroxy fatty acids accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial trifunctional protein deficiencies uncouple oxidative phosphorylation in heart mitochondriaJournal of Bioenergetics and Biomembranes, 2012
- Fatty Acid Accumulation and Resulting PPARActivation in Fibroblasts due to Trifunctional Protein DeficiencyPPAR Research, 2012
- Outcome in six patients with mitochondrial trifunctional protein disorders identified by newborn screeningMolecular Genetics and Metabolism, 2010
- Long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies induce oxidative stress in rat brainNeurochemistry International, 2010
- Mitochondrial fatty acid oxidation disorders: pathophysiological studies in mouse modelsJournal of Inherited Metabolic Disease, 2010
- Fatty acid oxidation disorders: outcome and long‐term prognosisJournal of Inherited Metabolic Disease, 2010
- General Mitochondrial Trifunctional Protein (TFP) Deficiency as a Result of Either α- or β-Subunit Mutations Exhibits Similar Phenotypes Because Mutations in Either Subunit Alter TFP Complex Expression and Subunit TurnoverPediatric Research, 2004
- Peripheral neuropathy, episodic myoglobinuria, and respiratory failure in deficiency of the mitochondrial trifunctional proteinMuscle & Nerve, 2003