Fuel utilization in patients with very long-chain acyl-coa dehydrogenase deficiency
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Fuel utilization in patients with very long-chain acyl-coa dehydrogenase deficiency. / ØRngreen, Mette C; Nørgaard, Mette; Sacchetti, Massimo; van Engelen, Baziel G M; Vissing, John; Charlot, Mette Gitz.
I: Annals of Neurology, Bind 56, Nr. 2, 01.08.2004, s. 279-83.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Fuel utilization in patients with very long-chain acyl-coa dehydrogenase deficiency
AU - ØRngreen, Mette C
AU - Nørgaard, Mette
AU - Sacchetti, Massimo
AU - van Engelen, Baziel G M
AU - Vissing, John
AU - Charlot, Mette Gitz
N1 - Copyright 2004 American Neurological Association
PY - 2004/8/1
Y1 - 2004/8/1
N2 - 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.
AB - 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.
U2 - http://dx.doi.org/10.1002/ana.20168
DO - http://dx.doi.org/10.1002/ana.20168
M3 - Journal article
VL - 56
SP - 279
EP - 283
JO - Annals of Neurology
JF - Annals of Neurology
SN - 0364-5134
IS - 2
ER -
ID: 34085619