Fuel utilization in patients with very long-chain acyl-coa dehydrogenase deficiency

Autor:	Mette C, ØRngreen, Mette G, Nørgaard, Massimo, Sacchetti, Baziel G M, van Engelen, John, Vissing
Rok vydání:	2004
Předmět:	Adult Blood Glucose Male Carbon Isotopes Acyl-CoA Dehydrogenase Long-Chain Fatty Acids Carbon Dioxide Middle Aged Gas Chromatography-Mass Spectrometry Oxygen Muscular Diseases Heart Rate Humans Insulin Lactic Acid Exercise
Zdroj:	Annals of neurology. 56(2)
ISSN:	0364-5134
Popis:	Fuel utilization in two adult patients with the myopathic form of very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency and five healthy subjects was investigated with stable isotopes during exercise at 50% of VO2max. The findings indicate that residual VLCAD activity in the patients is sufficient to maintain normal oxidation of fat at rest, but that fat oxidation rate cannot increase above basal levels during exercise. This can cause an energy deficit and intramuscular accumulation of fat intermediates that may induce the exercise-induced symptoms.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=pmid________::44fca04ab4dbe47d46861c3ef19f7e83 https://pubmed.ncbi.nlm.nih.gov/15293280 Zobrazit plný text záznamu Plný text