Impaired lipolysis in propionic acidemia: A new metabolic myopathy?
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ABSTRACT:
SUBMITTER: Storgaard JH
PROVIDER: S-EPMC7203654 | biostudies-literature | 2020 May
REPOSITORIES: biostudies-literature
Publications
Impaired lipolysis in propionic acidemia: A new metabolic myopathy?
Storgaard Jesper H JH Madsen Karen L KL Løkken Nicoline N Vissing John J van Hall Gerrit G Lund Allan M AM Ørngreen Mette C MC
JIMD reports 20200331 1
The objective of this study was to investigate the fat and carbohydrate metabolism in a patient with propionic acidemia (PA) during exercise by means of indirect calorimetry and stable isotope technique. A 34-year-old patient with PA performed a 30-minute submaximal cycle ergometer test. Data were compared to results from six gender- and age-matched healthy controls. Main findings are that the patient with PA had impaired lipolysis, blunted fatty acid oxidation, compensatory increase in carbohyd ...[more]
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