Experimental evidence for protein oxidative damage and altered antioxidant defense in patients with medium-chain acyl-CoA dehydrogenase deficiency

@article{Derks2014ExperimentalEF,
  title={Experimental evidence for protein oxidative damage and altered antioxidant defense in patients with medium-chain acyl-CoA dehydrogenase deficiency},
  author={Terry G. J. Derks and Catharina M. L. Touw and Graziela Schmitt Ribas and Giovana Brondani Biancini and Camila Simioni Vanzin and Giovanna Webster Negretto and Caroline Paula Mescka and Dirk-Jan Reijngoud and G. Peter A. Smit and Moacir Wajner and Carmen Regla Vargas},
  journal={Journal of Inherited Metabolic Disease},
  year={2014},
  volume={37},
  pages={783-789}
}
The objective of this study was to test whether macromolecule oxidative damage and altered enzymatic antioxidative defenses occur in patients with medium-chain acyl coenzyme A dehydrogenase (MCAD) deficiency. We performed a cross-sectional observational study of in vivo parameters of lipid and protein oxidative damage and antioxidant defenses in asymptomatic, nonstressed, MCAD-deficient patients and healthy controls. Patients were subdivided into three groups based on therapy: patients without… 
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21 Citations

Increased antioxidant response in medium-chain acyl-CoA dehydrogenase deficiency: does lipoic acid have a protective role?

It is raised the interesting hypothesis that increased PDC-bound lipoic acid, synthesized from accumulated octanoic acid in MCAD, may affect the cellular antioxidant pool in MCADD.

Oxidative damage in mitochondrial fatty acids oxidation disorders patients and the in vitro effect of l-carnitine on DNA damage induced by the accumulated metabolites.

Evidence that Oxidative Disbalance and Mitochondrial Dysfunction are Involved in the Pathophysiology of Fatty Acid Oxidation Disorders

Evidence from the scientific literature is presented on the role of oxidative damage and mitochondrial dysfunction in the pathogenesis of the most prevalent FAODs: medium-chain acyl-CoA dehydrogenase (MCAD), long-chain 3-hydroxyacyl- coA dehydrogensase (LCHAD) and very long- chain acyl

Recent Advances in the Pathophysiology of Fatty Acid Oxidation Defects: Secondary Alterations of Bioenergetics and Mitochondrial Calcium Homeostasis Caused by the Accumulating Fatty Acids

The present knowledge on disturbances of mitochondrial bioenergetics, calcium homeostasis, uncoupling of oxidative phosphorylation, and mitochondrial permeability transition induction provoked by the major fatty acids accumulating in prevalent FAOD are updated.

Understanding the role of OXPHOS dysfunction in the pathogenesis of ECHS1 deficiency

The clinical, biochemical and genetic features of all ECHS1‐deficient patients described to date are examined and the secondary OXPHOS defects associated with E CHS1 deficiency are considered and their possible contribution to disease pathogenesis is discussed.

Transcriptome analysis suggests a compensatory role of the cofactors coenzyme A and NAD+ in medium-chain acyl-CoA dehydrogenase knockout mice

It is discussed how metabolic adaptations in the liver may contribute to robustness in MCAD-KO mice and potentially also in asymptomatic human subjects with a complete loss of MCAD activity.

cis-4-Decenoic and decanoic acids impair mitochondrial energy, redox and Ca(2+) homeostasis and induce mitochondrial permeability transition pore opening in rat brain and liver: Possible implications for the pathogenesis of MCAD deficiency.

[An analysis of clinical characteristics and gene mutation in two patients with medium- and short-chain acyl-CoA dehydrogenase deficiency].

Screening tests for genetic metabolic diseases are recommended for children who have unexplained metabolic acidosis and hypoglycemia and genetic analyses of the ACADM and ACADS genes are helpful for the diagnosis of medium- and short-chain acyl-CoA dehydrogenase deficiency.

NDRG2 overexpression suppresses hepatoma cells survival during metabolic stress through disturbing the activation of fatty acid oxidation.

  • Tao PanMei Zhang Xia Li
  • Biology, Medicine
    Biochemical and biophysical research communications
  • 2017

DNA Damage and Oxidative Status of Full Term Babies Delivered by Spontaneous Vaginal Delivery and Caesarean Section

Objectives: At this study, it is aimed to research DNA damage and oxidative stress in infants with born timely normal spontaneous vaginal delivery (NSVD) and elective caesarean.Study Design: Healthy…

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