Impaired lipolysis in propionic acidemia: A new metabolic myopathy?

Jesper H Storgaard; Karen L Madsen; Nicoline Løkken; John Vissing; Gerrit van Hall; Allan M Lund; Mette C Ørngreen

doi:10.1002/jmd2.12113

Impaired lipolysis in propionic acidemia: A new metabolic myopathy?

Jesper H Storgaard, Karen L Madsen, Nicoline Løkken, John Vissing, Gerrit van Hall, Allan M Lund, Mette C Ørngreen

9 Citations (Scopus)

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.

Original language	English
Journal	JIMD Reports
Volume	53
Issue number	1
Pages (from-to)	16-21
Number of pages	6
ISSN	2192-8304
DOIs	https://doi.org/10.1002/jmd2.12113
Publication status	Published - May 2020

Keywords

Carbohydrate metabolism
Exercise metabolism
Fat metabolism
Metabolic myopathy
Organic aciduria
Propionic acidemia

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10.1002/jmd2.12113

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title = "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.",

keywords = "Carbohydrate metabolism, Exercise metabolism, Fat metabolism, Metabolic myopathy, Organic aciduria, Propionic acidemia",

author = "Storgaard, {Jesper H} and Madsen, {Karen L} and Nicoline L{\o}kken and John Vissing and {van Hall}, Gerrit and Lund, {Allan M} and {\O}rngreen, {Mette C}",

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year = "2020",

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doi = "10.1002/jmd2.12113",

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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

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.

KW - Carbohydrate metabolism

KW - Exercise metabolism

KW - Fat metabolism

KW - Metabolic myopathy

KW - Organic aciduria

KW - Propionic acidemia

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DO - 10.1002/jmd2.12113

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SP - 16

EP - 21

JO - JIMD Reports

JF - JIMD Reports

IS - 1

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Impaired lipolysis in propionic acidemia: A new metabolic myopathy?

Abstract

Keywords

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