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

  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},
  volume={527 1},
Metabolic Outcomes of Anaplerotic Dodecanedioic Acid Supplementation in Very Long Chain Acyl-CoA Dehydrogenase (VLCAD) Deficient Fibroblasts
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
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
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
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.


The Identification of a Succinyl-CoA Thioesterase Suggests a Novel Pathway for Succinate Production in Peroxisomes*
The identification of a highly specific succinyl-CoA thioesterase in peroxisomes strongly suggests that peroxISomal β-oxidation of dicarboxylic acids leads to formation of succinate, at least under certain conditions, and that ACOT4 and ACOT8 are responsible for the termination of β-Oxidation ofdicar boxylic amino acids of medium-chain length with the concomitant release of the corresponding free acids.
Dicarboxylic acids, an alternate fuel substrate in parenteral nutrition: an update.
Medium-chain DA have the peculiar characteristic of being water soluble due to the presence of two carboxylic terminal groups in the molecule, and they can be given by a peripheral vein as inorganic salts.
The microsomal dicarboxylyl-CoA synthetase.
It appears that the chain-length specificity of the handling of the Handling of dicarboxylic acids by this catabolic pathway parallels the pattern of the degradation of exogenous dicarusiclic acids in vivo.
In vitro studies on the oxidation of medium-chain dicarboxylic acids in rat liver.
Evidence is given to a pathway for medium-chained monocarboxylic acids (especially lauric acid and decanoic acid) through cytosolic omega-oxidation followed by activation, transport over the mitochondrial membrane and beta-oxidized to succinic acid.
Use of dicarboxylic acids in type 2 diabetes.
Study in animals and humans with type 2 diabetes showed that oral administration of sebacic acid improved glycaemic control, probably by enhancing insulin sensitivity, and reduced hepatic gluconeogenesis and glucose output, suggesting an improvement of energy utilization and 'metabolic flexibility'.
Compartmentation of Metabolism of the C12-, C9-, and C5-n-dicarboxylates in Rat Liver, Investigated by Mass Isotopomer Analysis
Dodecanedioate is a potential substrate for anaplerotic therapy of reperfusion injury and some inborn disorders of metabolism, and DODA contributes a substantial fraction to anaplerosis of the citric acid cycle.