Deregulation of mitochondrial functions provoked by long-chain fatty acid accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial permeability transition deficiencies in rat heart--mitochondrial permeability transition pore open (Q40499403)

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English	Deregulation of mitochondrial functions provoked by long-chain fatty acid accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial permeability transition deficiencies in rat heart--mitochondrial permeability transition pore open	scientific article

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Europe PubMed Central

21 September 2017

Deregulation of mitochondrial functions provoked by long-chain fatty acid accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial permeability transition deficiencies in rat heart--mitochondrial permeability transition pore open (English)

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Europe PubMed Central

21 September 2017

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Cristiane Cecatto

1

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3 June 2020

Fernanda H Hickmann

2

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3 June 2020

Alexandre U Amaral

4

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3 June 2020

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26 September 2015

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21 September 2017

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3 June 2020

282

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3 June 2020

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3 June 2020

4714-4726

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3 June 2020

Fatty acid oxidation disorders

1 reference

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21 January 2018

Neonatal screening for defects of the mitochondrial trifunctional protein

1 reference

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21 January 2018

Isolated mitochondrial long-chain ketoacyl-CoA thiolase deficiency resulting from mutations in the HADHB gene

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Early neonatal diagnosis of long-chain 3-hydroxyacyl coenzyme a dehydrogenase and mitochondrial trifunctional protein deficiencies.

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Peripheral neuropathy, episodic myoglobinuria, and respiratory failure in deficiency of the mitochondrial trifunctional protein.

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Intrauterine cardiomyopathy and cardiac mitochondrial proliferation in mitochondrial trifunctional protein (TFP) deficiency

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Mitochondrial trifunctional protein deficiency: a severe fatty acid oxidation disorder with cardiac and neurologic involvement

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Deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase: a cause of lethal myopathy and cardiomyopathy in early childhood

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Mitochondrial fatty acid oxidation disorders: clinical presentation of long-chain fatty acid oxidation defects before and after newborn screening

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Genetic and cellular modifiers of oxidative stress: what can we learn from fatty acid oxidation defects?

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Fatty Acid Accumulation and Resulting PPARα Activation in Fibroblasts due to Trifunctional Protein Deficiency

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Long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies induce oxidative stress in rat brain

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21 January 2018

Long-chain 3-hydroxy fatty acids accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial trifunctional protein deficiencies uncouple oxidative phosphorylation in heart mitochondria

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21 January 2018

Disturbance of mitochondrial energy homeostasis caused by the metabolites accumulating in LCHAD and MTP deficiencies in rat brain

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21 January 2018

Secondary respiratory chain defect in a boy with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: possible diagnostic pitfalls

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21 January 2018

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?

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21 January 2018

Disorders of mitochondrial fatty acyl-CoA beta-oxidation

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21 January 2018

The early-onset phenotype of mitochondrial trifunctional protein deficiency: a lethal disorder with multiple tissue involvement

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21 January 2018

Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to ?-subunit mutations

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21 January 2018

Properties of the permeability transition pore in mitochondria devoid of Cyclophilin D.

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21 January 2018

Inhibitory effects of adenine nucleotides on brain mitochondrial permeability transition

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21 January 2018

The mitochondrial permeability transition.

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21 January 2018

The molecular identity of the mitochondrial Ca2+ sequestration system

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21 January 2018

Mitochondrial permeability transition in Ca(2+)-dependent apoptosis and necrosis

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21 January 2018

Uncoupling, metabolic inhibition and induction of mitochondrial permeability transition in rat liver mitochondria caused by the major long-chain hydroxyl monocarboxylic fatty acids accumulating in LCHAD deficiency

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21 January 2018

Integrative genomics identifies MCU as an essential component of the mitochondrial calcium uniporter

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21 January 2018

A forty-kilodalton protein of the inner membrane is the mitochondrial calcium uniporter

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21 January 2018

The mitochondrial calcium uniporter complex: molecular components, structure and physiopathological implications

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21 January 2018

The mitochondrial permeability transition pore

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21 January 2018

The permeability transition pore as a Ca(2+) release channel: new answers to an old question

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21 January 2018

PINK1-associated Parkinson's disease is caused by neuronal vulnerability to calcium-induced cell death

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21 January 2018

Impairment of mitochondrial calcium handling in a mtSOD1 cell culture model of motoneuron disease

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21 January 2018

The mitochondrial permeability transition pore in motor neurons: involvement in the pathobiology of ALS mice

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21 January 2018

Role of mitochondrial dysfunction in the pathogenesis of Huntington's disease

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21 January 2018

Mitochondrial calcium, oxidative stress and apoptosis in a neurodegenerative disease model induced by 3-nitropropionic acid

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21 January 2018

Mitochondrial Ca2+ uptake contributes to buffering cytoplasmic Ca2+ peaks in cardiomyocytes

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21 January 2018

Opening of the mitochondrial permeability transition pore by uncoupling or inorganic phosphate in the presence of Ca2+ is dependent on mitochondrial-generated reactive oxygen species.

1 reference

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21 January 2018

Mitochondrial permeability transition and oxidative stress

1 reference

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21 January 2018

Regulation of Ca 2+ release from mitochondria by the oxidation-reduction state of pyridine nucleotides

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21 January 2018

Physiological roles of nicotinamide nucleotide transhydrogenase

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21 January 2018

The redox state of endogenous pyridine nucleotides can determine both the degree of mitochondrial oxidative stress and the solute selectivity of the permeability transition pore.

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21 January 2018

Fatty acid circuit as a physiological mechanism of uncoupling of oxidative phosphorylation

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Pathophysiology of fatty acid oxidation disorders and resultant phenotypic variability.

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The effect of fasting, long-chain triglyceride load and carnitine load on plasma long-chain acylcarnitine levels in mitochondrial very long-chain acyl-CoA dehydrogenase deficiency

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Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death

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Mitochondrial ATP-sensitive K+ channel opening decreases reactive oxygen species generation

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21 January 2018

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Mechanism for the uncoupling of oxidative phosphorylation by juliprosopine on rat brain mitochondria.

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Identifiers

10.1111/FEBS.13526

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26408230%20AND%20SRC:MED&resulttype=core&format=json

3 June 2020

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26408230%20AND%20SRC:MED&resulttype=core&format=json

3 June 2020

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