Mitochondrial dysfunction in fatty acid oxidation disorders: insights from human and animal studies

Wajner, Moaçir; Amaral, Alexandre Umpierrez

doi:10.1042/bsr20150240

Cited by 160 publications

(129 citation statements)

References 127 publications

(119 reference statements)

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“…There is a litany of diseases involving disruption to fatty-acid metabolism including metabolic cardiomyopathy in which carnitine transporter is impaired. Emerging evidence of mitochondrial dysfunction in other characterized diseases of fatty acid oxidation disorders involves changes in carnitine dynamics (Sharma and Black, 2009, Wajner and Amaral, 2016). Iron-responsive element-binding protein 2 (IRP2) has been identified as a leading candidate for COPD susceptibility gene in humans based on genome-wide association studies (DeMeo et al, 2009).…”

Section: Mitochondrial Stress Responsementioning

confidence: 99%

Mitochondrial redox system, dynamics, and dysfunction in lung inflammaging and COPD

Lerner

Sundar

Rahman

2016

The International Journal of Biochemistry & Cell Biology

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Myriad forms of endogenous and environmental stress will disrupt mitochondrial function by impacting critical processes in mitochondrial homeostasis such as mitochondrial redox system, oxidative phosphorylation, biogenesis, and mitophagy. External stressors that interfere with the steady state activity of mitochondrial functions are generally associated with an increase in reactive oxygen species, inflammatory response, and induction of cellular senescence (inflammaging) via mitochondrial damage associated molecular patterns (DAMPS) which are the key events in the pathogenesis of chronic obstructive pulmonary disease (COPD) and its exacerbations. In this review, we highlight the primary mitochondrial quality control mechanisms that are influenced by oxidative stress/redox system, including role of mitochondria during inflammation and cellular senescence, and how mitochondrial dysfunction contributes to the pathogenesis of COPD and its exacerbations via pathogenic stimuli.

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Section: Mitochondrial Stress Responsementioning

confidence: 99%

Mitochondrial redox system, dynamics, and dysfunction in lung inflammaging and COPD

Lerner

Sundar

Rahman

2016

The International Journal of Biochemistry & Cell Biology

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“…As in other fatty acid oxidation defects, a combination of hypoglycemia and toxicity of fatty acid intermediates can result in liver, brain, and heart injury. There is evidence of more general mitochondrial dysfunction in these disorders, including generation of reactive oxygen species and calcium imbalance (Wajner and Amaral 2015). During catabolic circumstances such as prolonged fasting, stress, illness, and surgery, metabolic derangement can occur, resulting in hypoglycemia, myopathies (including cardiomyopathy), metabolic acidosis, and rhabdomyolysis (Leslie et al 1993;Redshaw and Stewart 2014).…”

Section: Introductionmentioning

confidence: 99%

Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency and Perioperative Management in Adult Patients

et al. 2016

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Surgery and anesthesia pose a threat to patients with very long-chain acyl-CoA dehydrogenase deficiency (VLCADD), because prolonged fasting, stress, and pain are known risk factors for the induction of metabolic derangement. The optimal perioperative management in these patients is unknown and the use of volatile agents and agents dissolved in fatty acids has been related to postoperative metabolic complications. However, the occurrence of metabolic derangement is multifactorial and depends, amongst others, on the severity of the mutation and residual enzyme activity. Current guidelines suggest avoiding both volatile anesthetics as well as propofol, which seriously limits the options for providing safe anesthesia. Therefore, we reviewed the available literature on the perioperative management of patients with VLCADD. We concluded that the use of some medications, such as volatile anesthetics, in patients with VLCADD might be wrongfully avoided and could in fact prevent metabolic derangement by the adequate suppression of pain and stress during surgery. We will illustrate this with a case report of an adult VLCADD patient undergoing minor surgery. Besides the use of remifentanil, anesthesia was uneventfully maintained with the use of sevoflurane, a volatile agent, and continuous glucose infusion. The patient was monitored with a continuous glucose meter and creatinine kinase measurements.

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“…Natomiast dodatek tłuszczu do paszy o LN 50 mEq O 2 /kg powoduje zmiany patomorfologiczne w wątrobie, nerkach i mięśniach szkieletowych o charakterze uszkadzającym i adaptacyjnym (3,9,16,21,(51)(52)(53). Ponadto zmiany te nasilają się wraz ze wzrostem stopnia utlenienia tłuszczu (52,53,63,64).…”

Section: Tab 1 Objawy Kliniczne I Zmiany Anatomopatologiczne U Drobunclassified

Consequences of using rations with oxidised fats in poultry feeding

Grudzień¹,

Szarek²,

Babińska³

et al. 2018

Medycyna Weterynaryjna

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SummaryFat is an important component of feed rations for poultry as it improves the growth and laying parameters, which translates into better economic outcomes of animal production. The quality of this nutrient is determined by its freshness. It was assumed that the addition of oxidized fat with a peroxide value below 6 mEq O 2 /kg in the ration does not trigger any clinical symptoms or morphologic changes in the internal organs in poultry. Fat with a higher degree of oxidation is harmful to animal health and thus reduces the performance. The products of hydrolysis and oxidation of higher fatty acids demonstrate cytotoxic effects and lead to metabolic disturbances. Consequently, this results in morphological changes of both a destructive and adaptive nature. These changes are mainly observed in the gastrointestinal tract, liver, kidneys, skin, skeletal muscles and the heart in different avian species. Furthermore, poultry becomes more susceptible to viral and bacterial infections. Disease cases resulting from feeding oxidized fats to poultry often become a hot spot and a source of conflict between a feed provider and a customer. They also require veterinary attendance and interventions. In such circumstances, the first stage of treatment always consists in a change in the current feed for a novel, good quality feed mix. In addition, detoxifying, hepatoprotective and protective medications are warranted.

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Mitochondrial dysfunction in fatty acid oxidation disorders: insights from human and animal studies

Cited by 160 publications

References 127 publications

Mitochondrial redox system, dynamics, and dysfunction in lung inflammaging and COPD

Mitochondrial redox system, dynamics, and dysfunction in lung inflammaging and COPD

Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency and Perioperative Management in Adult Patients

Consequences of using rations with oxidised fats in poultry feeding

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