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

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

SN - 0364-5134

VL - 56

SP - 279

EP - 283

JO - Annals of Neurology

JF - Annals of Neurology

IS - 2

ER -