scholarly journals LONG-CHAIN SPECIFIC ENZYME FROM MEDIUM-CHAIN ACYL-COA DEHYDROGENASE

1994 ◽  
pp. 327-330
Keyword(s):  
Specific Enzyme ◽  
Medium Chain ◽  
Chain Acyl ◽  
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

scholarly journals Medium-Chain Acyl-CoA Dehydrogenase Deficiency in an Infant with Dilated Cardiomyopathy

2009 ◽  
Vol 2009 ◽  
pp. 1-3 ◽  
Author(s):  
Marcello Marcì ◽  
Patrizia Ajovalasit
Keyword(s):  
Fatty Acid ◽  
Dilated Cardiomyopathy ◽  
Dehydrogenase Deficiency ◽  
Acid Oxidation ◽  
Inherited Disorders ◽  
Mitochondrial Fatty Acid Oxidation ◽  
Medium Chain ◽  
Mitochondrial Fatty Acid ◽  
Chain Acyl ◽  
Long Chain

We report about an infant affected by dilated cardiomyopathy (CMP) in whom metabolic investigations evidenced medium-chain-acyl-CoA dehydrogenase deficiency (MCADD), that is one of three types of inherited disorders of mitochondrial fatty-acid -oxidation. Long-chain and very long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficits are recognized as responsible of hypertrophic or, less frequently, dilated cardiomyopathy (CMP) in childhood. Otherwise, to our knowledge, no case of MCADD associated to dilated CMP has been reported in literature.

Hypoglycemia, Hypotonia, and Cardiomyopathy: The Evolving Clinical Picture of Long-Chain Acyl-CoA Dehydrogenase Deficiency

PEDIATRICS ◽  
1991 ◽  
Vol 87 (3) ◽  
pp. 328-333 ◽  
Author(s):  
William R. Treem ◽  
Jeffrey S. Hyams ◽  
Charles A. Stanley ◽  
Daniel E. Hale ◽  
Harris B. Leopold
Keyword(s):  
Fatty Acid ◽  
Fatty Acid Oxidation ◽  
Dehydrogenase Deficiency ◽  
Response To Treatment ◽  
Acid Oxidation ◽  
Medium Chain ◽  
Chain Acyl ◽  
History Of ◽  
Related Enzyme ◽  
Long Chain

Inherited defects in fatty acid oxidation, which have been described and diagnosed with increasing frequency in the last decade, are most commonly attributed to a deficiency in the activity of medium-chain acyl-CoA dehydrogenase. Few cases of the related enzyme defect of long-chain acyl-CoA dehydrogenase activity have been reported. An infant with documented long-chain acyl-CoA dehydrogenase deficiency is described with a detailed metabolic profile, long-term clinical follow-up, and response to treatment. This patient is compared with the seven previously published cases of this disorder in order to stress the unique features of the initial presentation, more subtle late manifestations of the disease, and clinical and biochemical differentiation from the more common medium-chain acyl-CoA dehydrogenase deficiency. This report stresses the enlarging spectrum of the clinical presentation and natural history of this defect in fatty acid oxidation.

Medium-chain triglycerides impair lipid metabolism and induce hepatic steatosis in very long-chain acyl-CoA dehydrogenase (VLCAD)-deficient mice

2010 ◽  
Vol 101 (1) ◽  
pp. 40-47 ◽  
Author(s):  
Sara Tucci ◽  
Sonja Primassin ◽  
Frank Ter Veld ◽  
Ute Spiekerkoetter
Keyword(s):  
Lipid Metabolism ◽  
Hepatic Steatosis ◽  
Medium Chain Triglycerides ◽  
Medium Chain ◽  
Chain Acyl ◽  
Deficient Mice ◽  
Long Chain

Spectroscopic analysis of the interaction of rat liver short-chain, medium-chain, and long-chain acyl coenzyme A dehydrogenases with acyl coenzyme A substrates

Biochemistry ◽  
1985 ◽  
Vol 24 (25) ◽  
pp. 7192-7199 ◽  
Author(s):  
Yasuyuki Ikeda ◽  
Kazuko Okamura-Ikeda ◽  
Kay Tanaka
Keyword(s):  
Rat Liver ◽  
Spectroscopic Analysis ◽  
Coenzyme A ◽  
Short Chain ◽  
Medium Chain ◽  
Chain Acyl ◽  
Acyl Coenzyme A ◽  
Long Chain
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