Impaired lipolysis in propionic acidemia: A new metabolic myopathy?
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Impaired lipolysis in propionic acidemia : A new metabolic myopathy? / Storgaard, Jesper H; Madsen, Karen L; Løkken, Nicoline; Vissing, John; van Hall, Gerrit; Lund, Allan M; Ørngreen, Mette C.
I: JIMD Reports, Bind 53, Nr. 1, 05.2020, s. 16-21.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Impaired lipolysis in propionic acidemia
T2 - A new metabolic myopathy?
AU - Storgaard, Jesper H
AU - Madsen, Karen L
AU - Løkken, Nicoline
AU - Vissing, John
AU - van Hall, Gerrit
AU - Lund, Allan M
AU - Ørngreen, Mette C
N1 - © 2020 The Authors. Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM.
PY - 2020/5
Y1 - 2020/5
N2 - 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.
AB - 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.
U2 - 10.1002/jmd2.12113
DO - 10.1002/jmd2.12113
M3 - Journal article
C2 - 32395405
VL - 53
SP - 16
EP - 21
JO - JIMD Reports
JF - JIMD Reports
SN - 2192-8304
IS - 1
ER -
ID: 249164654