scholarly journals Molecular evolution and substrate specificity of acyl-CoA dehydrogenases: Chimaeric ‘medium/long’ chain-specific enzyme from medium-chain acyl-CoA dehydrogenase

1996 ◽  
Vol 24 (1) ◽  
pp. 105-110 ◽  
Author(s):  
A. Nandy ◽  
B. Küchler ◽  
S. Ghisla
Keyword(s):  
Molecular Evolution ◽  
Substrate Specificity ◽  
Specific Enzyme ◽  
Medium Chain ◽  
Chain Acyl ◽  
Acyl Coa Dehydrogenases ◽  
Long Chain

Human long chain, very long chain and medium chain acyl-CoA dehydrogenases are specific for the S-enantiomer of 2- methylpentadecanoyl-CoA

1998 ◽  
Vol 1390 (3) ◽  
pp. 333-338 ◽  
Author(s):  
Kevin P Battaile ◽  
Martha McBurney ◽  
Paul P Van Veldhoven ◽  
Jerry Vockley
Keyword(s):  
Medium Chain ◽  
Chain Acyl ◽  
Acyl Coa Dehydrogenases ◽  
Long Chain

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

2020 ◽  
Vol 11 ◽  
Author(s):  
Alexandre Umpierrez Amaral ◽  
Moacir Wajner
Keyword(s):  
Fatty Acids ◽  
Fatty Acid ◽  
Fatty Acid Oxidation ◽  
Calcium Homeostasis ◽  
Acid Oxidation ◽  
Cultured Fibroblasts ◽  
Medium Chain ◽  
Chain Acyl ◽  
Energy Deprivation ◽  
Long Chain

Deficiencies of medium-chain acyl-CoA dehydrogenase, mitochondrial trifunctional protein, isolated long-chain 3-hydroxyacyl-CoA dehydrogenase, and very long-chain acyl-CoA dehydrogenase activities are considered the most frequent fatty acid oxidation defects (FAOD). They are biochemically characterized by the accumulation of medium-chain, long-chain hydroxyl, and long-chain fatty acids and derivatives, respectively, in tissues and biological fluids of the affected patients. Clinical manifestations commonly include hypoglycemia, cardiomyopathy, and recurrent rhabdomyolysis. Although the pathogenesis of these diseases is still poorly understood, energy deprivation secondary to blockage of fatty acid degradation seems to play an important role. However, recent evidence indicates that the predominant fatty acids accumulating in these disorders disrupt mitochondrial functions and are involved in their pathophysiology, possibly explaining the lactic acidosis, mitochondrial morphological alterations, and altered mitochondrial biochemical parameters found in tissues and cultured fibroblasts from some affected patients and also in animal models of these diseases. In this review, we will update 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. It is emphasized that further in vivo studies carried out in tissues from affected patients and from animal genetic models of these disorders are necessary to confirm the present evidence mostly achieved from in vitro experiments.

scholarly journals Sex‐specific perturbation of complex lipids in response to medium‐chain fatty acids in very long‐chain acyl‐CoA dehydrogenase deficiency

FEBS Journal ◽  
2020 ◽  
Vol 287 (16) ◽  
pp. 3511-3525 ◽  
Author(s):  
Khaled I. Alatibi ◽  
Zeinab Wehbe ◽  
Ute Spiekerkoetter ◽  
Sara Tucci
Keyword(s):  
Fatty Acids ◽  
Dehydrogenase Deficiency ◽  
Medium Chain ◽  
Medium Chain Fatty Acids ◽  
Chain Acyl ◽  
Complex Lipids ◽  
Long Chain ◽  
Chain Fatty Acids

Pre-exercise medium-chain triglyceride application prevents acylcarnitine accumulation in skeletal muscle from very-long-chain acyl-CoA-dehydrogenase-deficient mice

2010 ◽  
Vol 33 (3) ◽  
pp. 237-246 ◽  
Author(s):  
Sonja Primassin ◽  
Sara Tucci ◽  
Diran Herebian ◽  
Annette Seibt ◽  
Lars Hoffmann ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Medium Chain Triglyceride ◽  
Medium Chain ◽  
Chain Acyl ◽  
Deficient Mice ◽  
Long Chain
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