Impaired lipolysis in propionic acidemia: A new metabolic myopathy?

Autor: Jesper H. Storgaard, Karen L. Madsen, Nicoline Løkken, John Vissing, Gerrit vanHall, Allan M. Lund, Mette C. Ørngreen
Jazyk: angličtina
Rok vydání: 2020
Předmět:
Zdroj: JIMD Reports, Vol 53, Iss 1, Pp 16-21 (2020)
Druh dokumentu: article
ISSN: 2192-8312
DOI: 10.1002/jmd2.12113
Popis: 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.
Databáze: Directory of Open Access Journals