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Impaired lipolysis in propionic acidemia: A new metabolic myopathy?


ABSTRACT: 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 carbohydrate utilization, and low work capacity. Our findings indicate that PA should be added to the list of metabolic myopathies.

SUBMITTER: Storgaard JH 

PROVIDER: S-EPMC7203654 | biostudies-literature | 2020 May

REPOSITORIES: biostudies-literature

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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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