Impaired lipolysis in propionic acidemia: A new metabolic myopathy?. Issue 1 (31st March 2020)

• Record Type: Journal Article
• Authors: Storgaard, Jesper H.; Madsen, Karen L.; Løkken, Nicoline; Vissing, John; van Hall, Gerrit; Lund, Allan M.; Ørngreen, Mette C.
• Is Part Of: JIMD reports. Volume 53:Issue 1(2020)
• Page Start: 16
• Page End: 21
• Publication Date: 2020-03-31
• Subjects: carbohydrate metabolism -- exercise metabolism -- fat metabolism -- metabolic myopathy -- organic aciduria -- propionic acidemia; Metabolism, Inborn errors of -- Periodicals; Metabolism -- Disorders -- Periodicals; 616.39042
• DOI: 10.1002/jmd2.12113
• Languages: English
• ISSNs: 2192-8304

Abstract

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.