Role of mitochondrial acyl-CoA dehydrogenases in the metabolism of dicarboxylic fatty acids.

@article{Bharathi2020RoleOM,
  title={Role of mitochondrial acyl-CoA dehydrogenases in the metabolism of dicarboxylic fatty acids.},
  author={Sivakama S. Bharathi and Yuxun Zhang and Zhenwei Gong and Radhika Muzumdar and Eric S. Goetzman},
  journal={Biochemical and biophysical research communications},
  year={2020},
  volume={527 1},
  pages={
          162-166
        }
}
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Metabolic Outcomes of Anaplerotic Dodecanedioic Acid Supplementation in Very Long Chain Acyl-CoA Dehydrogenase (VLCAD) Deficient Fibroblasts
TLDR
Dodecanedioic acid supplementation replenishes the Krebs cycle by increasing the succinate pool, attenuates glycolytic flux, and reduces levels of toxic very long-chain acylcarnitines.
The Mystery of Extramitochondrial Proteins Lysine Succinylation
  • C. Chinopoulos
  • Medicine
    International journal of molecular sciences
  • 2021
TLDR
The discovery that glia in the adult human brain lack subunits of both alpha-ketoglutarate dehydrogenase complex and succinate-CoA ligase—thus being unable to produce succinyl- CoA in the matrix—and yet exhibit robust pancellular lysine succinylation, is highlighted.
Long‐chain fatty acid oxidation and respiratory complex I deficiencies distinguish Barth Syndrome from idiopathic pediatric cardiomyopathy
TLDR
Distinct shifts in the expression of long‐chain fatty acid oxidation enzymes and the tissue acyl‐CoA profile of BTHS hearts suggest a specific block in mitochondrial fatty acids oxidation upstream of the conventional matrix beta‐oxidation cycle, which may be compensated for by a greater reliance upon peroxisomal fatty Acid oxidation and the catabolism of ketones, amino acids, and pyruvate to meet cardiac energy demands.
Mitochondrial Lipid Homeostasis at the Crossroads of Liver and Heart Diseases
TLDR
Analysis of available genetic data suggests that the altered operation of fatty-acid β-oxidation in liver mitochondria is the key process, connecting NAFLD-mediated dyslipidemia and elevated CVD risk.

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