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Fatty Acid Oxidation Disorders
Annual Review of Physiology, 2002▪ Abstract Genetic disorders of mitochondrial fatty acid β-oxidation have been recognized within the last 20 years as important causes of morbidity and mortality, highlighting the physiological significance of fatty acids as an energy source. Although the mammalian mitochondrial fatty acid-oxidizing system was recognized at the beginning of the last ...
Piero, Rinaldo +2 more
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Mitochondrial Fatty-Acid Oxidation Disorders
Seminars in Pediatric Neurology, 2008Inherited defects in mitochondrial fatty-acid beta-oxidation comprise a group of at least 12 diseases characterized by distinct enzyme or transporter deficiencies. Most of these diseases have a variable age of onset and clinical severity. Symptoms are often episodic and associated with mild viral illness, physiologic stress, or prolonged exercise that ...
Michelle, Kompare, William B, Rizzo
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Abnormalities of Fatty Acid Oxidation
New England Journal of Medicine, 1988Inborn errors involving the oxidative metabolism of fatty acids represent a newly recognized area of human disease.
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Plasma free fatty acids in mitochondrial fatty acid oxidation defects
Clinica Chimica Acta, 1997Plasma free fatty acid profiles from patients suffering from various mitochondrial beta-oxidation deficiencies were analyzed by gas chromatography-mass spectrometry. cis-4-Decenoic acid (10:1n-6) in medium-chain acyl-CoA dehydrogenase deficiency and cis-5-tetradecenoic acid (14:1n-9) in very-long-chain and 3-hydroxy-long chain acyl-CoA dehydrogenase ...
G, Martínez +7 more
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Fatty acid oxidation and epilepsy
Epilepsy Research, 2012The ketogenic diet (KD) is a high-fat and low carbohydrate diet with an established efficacy to treat refractory epilepsy. Lipids, particularly fatty acids, are nutrients which provide the most important part of the caloric intake under the KD. It has been suggested that the 'high-fat' component of the KD plays a role in its anticonvulsant properties ...
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Inhibitors of fatty acid oxidation
Life Sciences, 1987This review discusses inhibitors of fatty acid oxidation for which sites and mechanisms of inhibition are reasonably well understood. Included in this review are hypoglycin, an inhibitor of butyryl-CoA dehydrogenase (EC 1.3.99.2), 4-pentenoic acid, 2-bromooctanoic acid, and 4-bromocrotonic acid all of which inhibit mitochondrial thiolases (EC 2.3.1.9 ...
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Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1991
Dans cette revue generale on essaie de mettre en evidence les progres realises dans l'etude de la β oxydation des acides gras. On traite l'activation de l'internalisation des acides gras au niveau des cellules animales; on developpe la β oxydation au niveau de la mitochondrie, son activation, sa regulation et son inhibition.
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Dans cette revue generale on essaie de mettre en evidence les progres realises dans l'etude de la β oxydation des acides gras. On traite l'activation de l'internalisation des acides gras au niveau des cellules animales; on developpe la β oxydation au niveau de la mitochondrie, son activation, sa regulation et son inhibition.
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Disorders of fatty acid oxidation
2013Recognition of fatty acid oxidation (FAO) disorders is important for the pediatric neurologist as they present with a spectrum of clinical disorders, including progressive lipid storage myopathy, recurrent myoglobinuria, neuropathy, progressive cardiomyopathy, recurrent hypoglycemic hypoketotic encephalopathy or Reye-like syndrome, seizures, and mental
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Journal of the American Oil Chemists' Society, 1989
The history of allene oxides in organic chemistry is short. It goes back t o 1968, when Camp and Greene were able t o isolate and characterize an allene oxide which they obta ined b y monoepoxidation of 1,3-di-t-butylallene with mchloroperbenzoic acid (1).
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The history of allene oxides in organic chemistry is short. It goes back t o 1968, when Camp and Greene were able t o isolate and characterize an allene oxide which they obta ined b y monoepoxidation of 1,3-di-t-butylallene with mchloroperbenzoic acid (1).
openaire +1 more source