Abstract
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.
Copyright 2004 American Neurological Association
Publication types
-
Comparative Study
-
Research Support, Non-U.S. Gov't
MeSH terms
-
Acyl-CoA Dehydrogenase, Long-Chain / deficiency*
-
Adult
-
Blood Glucose / metabolism
-
Carbon Dioxide / blood
-
Carbon Isotopes / blood
-
Exercise / physiology
-
Fatty Acids / metabolism*
-
Gas Chromatography-Mass Spectrometry / methods
-
Heart Rate / physiology
-
Humans
-
Insulin / blood
-
Lactic Acid / blood
-
Male
-
Middle Aged
-
Muscular Diseases / enzymology*
-
Muscular Diseases / metabolism
-
Muscular Diseases / physiopathology
-
Oxygen / blood
Substances
-
Blood Glucose
-
Carbon Isotopes
-
Fatty Acids
-
Insulin
-
Carbon Dioxide
-
Lactic Acid
-
Acyl-CoA Dehydrogenase, Long-Chain
-
Oxygen