Ontology highlight
ABSTRACT:
SUBMITTER: Diekman EF
PROVIDER: S-EPMC4755596 | biostudies-literature | 2016
REPOSITORIES: biostudies-literature
Diekman E F EF Visser G G Schmitz J P J JP Nievelstein R A J RA de Sain-van der Velden M M Wardrop M M Van der Pol W L WL Houten S M SM van Riel N A W NA Takken T T Jeneson J A L JA
PloS one 20160216 2
Rhabdomyolysis is common in very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) and other metabolic myopathies, but its pathogenic basis is poorly understood. Here, we show that prolonged bicycling exercise against a standardized moderate workload in VLCADD patients is associated with threefold bigger changes in phosphocreatine (PCr) and inorganic phosphate (Pi) concentrations in quadriceps muscle and twofold lower changes in plasma acetyl-carnitine levels than in healthy subjects. This r ...[more]