Impaired lipolysis in propionic acidemia: A new metabolic myopathy?

Research output: Contribution to journal › Journal article › Research › peer-review

Standard

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.

In: JIMD Reports, Vol. 53, No. 1, 05.2020, p. 16-21.

Research output: Contribution to journal › Journal article › Research › peer-review

Harvard

Storgaard, JH, Madsen, KL, Løkken, N, Vissing, J, van Hall, G, Lund, AM & Ørngreen, MC 2020, 'Impaired lipolysis in propionic acidemia: A new metabolic myopathy?', JIMD Reports, vol. 53, no. 1, pp. 16-21. https://doi.org/10.1002/jmd2.12113

APA

Storgaard, J. H., Madsen, K. L., Løkken, N., Vissing, J., van Hall, G., Lund, A. M., & Ørngreen, M. C. (2020). Impaired lipolysis in propionic acidemia: A new metabolic myopathy? JIMD Reports, 53(1), 16-21. https://doi.org/10.1002/jmd2.12113

Vancouver

Storgaard JH, Madsen KL, Løkken N, Vissing J, van Hall G, Lund AM et al. Impaired lipolysis in propionic acidemia: A new metabolic myopathy? JIMD Reports. 2020 May;53(1):16-21. https://doi.org/10.1002/jmd2.12113

Author

Storgaard, Jesper H ; Madsen, Karen L ; Løkken, Nicoline ; Vissing, John ; van Hall, Gerrit ; Lund, Allan M ; Ørngreen, Mette C. / Impaired lipolysis in propionic acidemia : A new metabolic myopathy?. In: JIMD Reports. 2020 ; Vol. 53, No. 1. pp. 16-21.

Bibtex

@article{c4fa7a00d261472ab5f96f345af41a86,
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.",
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}",
note = "{\textcopyright} 2020 The Authors. Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM.",
year = "2020",
month = may,
doi = "10.1002/jmd2.12113",
language = "English",
volume = "53",
pages = "16--21",
journal = "JIMD Reports",
issn = "2192-8304",
publisher = "Springer Berlin",
number = "1",

}

RIS

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