Alterations of Fat and Ketone Body Metabolism in <scp>ALS</scp> and <scp>SMA</scp>—A Prospective Observational Study
Our data suggest that already during early disease stages, ALS patients produce ketone bodies to compensate for the energy deficit. In SMA, on the other hand, the persistence of ketogenesis may indicate an upregulation of all available metabolic pathways for energy production due to the disturbance of fatty acid utilization. Therefore, the application of additional sources of energy, such as ketone bodies, might constitute a promising therapeutic option in both diseases.
Authors
- Linda L. Jagodzinski (ORCID: https://orcid.org/0000-0003-3142-8643)
- Claudia D. Wurster (ORCID: https://orcid.org/0000-0003-0411-9968)
- Sebastian Michels (ORCID: https://orcid.org/0000-0002-9265-9034)
- Alexander N.R. Weber (ORCID: https://orcid.org/0000-0002-8627-7056)
- Lisa Richter (ORCID: https://orcid.org/0000-0002-0769-619X)
- Jens Dreyhaupt (ORCID: https://orcid.org/0000-0001-8057-161X)
- Zeynep Elmas (ORCID: https://orcid.org/0000-0002-4092-3155)
- Željko Uzelac (ORCID: https://orcid.org/0000-0003-2479-2025)
- Joachim Schuster (ORCID: https://orcid.org/0000-0002-9577-9541)
- Johannes Dorst (ORCID: https://orcid.org/0000-0001-6352-0909)
- Christine Herrmann
Institutions
- Universität Ulm (DE)
- German Center for Neurodegenerative Diseases (DE)
- Zimmer Biomet (Germany) (DE)
Publication Details
- Journal
- European Journal of Neurology
- Published
- 2025-04-01
- DOI
- https://doi.org/10.1111/ene.70132
- Citations
- 2
- Primary Topic
- Neurogenetic and Muscular Disorders Research
- Type
- article
- Field-Weighted Citation Impact
- 3.25