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Live cell imaging of ATP levels reveals metabolic compartmentalization within motoneurons and early metabolic changes in FUS ALS motoneurons

Zimyanin, Vitaly L. (author)
Department of Molecular Physiology and Biological Physics, School of Medicine, University of Virginia, VA, Charlottesville, United States; Center for Membrane and Cell Physiology, School of Medicine, University of Virginia, VA, Charlottesville, United States; Department of Neurology, Technische Universität Dresden, Dresden, Germany
Pielka, Anna-Maria (author)
Translational Neurodegeneration Section, “Albrecht Kossel”, Department of Neurology, University Medical Center Rostock, University of Rostock, Rostock, Germany
Glaß, Hannes (author)
Translational Neurodegeneration Section, “Albrecht Kossel”, Department of Neurology, University Medical Center Rostock, University of Rostock, Rostock, Germany
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Japtok, Julia (author)
Department of Neurology, Technische Universität Dresden, Dresden, Germany
Großmann, Dajana (author)
Translational Neurodegeneration Section, “Albrecht Kossel”, Department of Neurology, University Medical Center Rostock, University of Rostock, Rostock, Germany
Martin, Melanie (author)
Institute of Physiology, Technische Universität Dresden, Dresden, Germany
Deussen, Andreas (author)
Institute of Physiology, Technische Universität Dresden, Dresden, Germany
Szewczyk, Barbara (author)
Translational Neurodegeneration Section, “Albrecht Kossel”, Department of Neurology, University Medical Center Rostock, University of Rostock, Rostock, Germany
Deppmann, Chris (author)
Department of Biology, Graduate School of Arts and Sciences, University of Virginia, VA, Charlottesville, United States
Zunder, Eli (author)
Department of Biomedical Engineering, School of Medicine, University of Virginia, VA, Charlottesville, United States
Andersen, Peter M., 1962- (author)
Umeå universitet,Neurovetenskaper
Boeckers, Tobias M. (author)
Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE), Ulm Site, Ulm, Germany; Institute for Anatomy and Cell Biology, Ulm University, Ulm, Germany
Sterneckert, Jared (author)
Centre for Regenerative Therapie, Technische Universität Dresden, Dresden, Germany; Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
Redemann, Stefanie (author)
Department of Molecular Physiology and Biological Physics, School of Medicine, University of Virginia, VA, Charlottesville, United States; Center for Membrane and Cell Physiology, School of Medicine, University of Virginia, VA, Charlottesville, United States; Department of Cell Biology, School of Medicine, University of Virginia, VA, Charlottesville, United States
Storch, Alexander (author)
Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE) Rostock/Greifswald, Rostock, Germany; Center for Transdisciplinary Neurosciences Rostock (CTNR), University Medical Centre, University of Rostock, Rostock, Germany; Department of Neurology, University of Rostock, Rostock, Germany
Hermann, Andreas (author)
Translational Neurodegeneration Section, “Albrecht Kossel”, Department of Neurology, University Medical Center Rostock, University of Rostock, Rostock, Germany; Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE) Rostock/Greifswald, Rostock, Germany; Center for Transdisciplinary Neurosciences Rostock (CTNR), University Medical Centre, University of Rostock, Rostock, Germany
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 (creator_code:org_t)
MDPI, 2023
2023
English.
In: Cells. - : MDPI. - 2073-4409. ; 12:10
Related links:
https://doi.org/10.3...
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https://umu.diva-por... (primary) (Raw object)
https://urn.kb.se/re...
https://doi.org/10.3...
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  • Journal article (peer-reviewed)
Abstract Subject headings
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  • Motoneurons are one of the most energy-demanding cell types and a primary target in Amyotrophic lateral sclerosis (ALS), a debilitating and lethal neurodegenerative disorder without currently available effective treatments. Disruption of mitochondrial ultrastructure, transport, and metabolism is a commonly reported phenotype in ALS models and can critically affect survival and the proper function of motor neurons. However, how changes in metabolic rates contribute to ALS progression is not fully understood yet. Here, we utilize hiPCS-derived motoneuron cultures and live imaging quantitative techniques to evaluate metabolic rates in fused in sarcoma (FUS)-ALS model cells. We show that differentiation and maturation of motoneurons are accompanied by an overall upregulation of mitochondrial components and a significant increase in metabolic rates that correspond to their high energy-demanding state. Detailed compartment-specific live measurements using a fluorescent ATP sensor and FLIM imaging show significantly lower levels of ATP in the somas of cells carrying FUS-ALS mutations. These changes lead to the increased vulnerability of diseased motoneurons to further metabolic challenges with mitochondrial inhibitors and could be due to the disruption of mitochondrial inner membrane integrity and an increase in its proton leakage. Furthermore, our measurements demonstrate heterogeneity between axonal and somatic compartments, with lower relative levels of ATP in axons. Our observations strongly support the hypothesis that mutated FUS impacts the metabolic states of motoneurons and makes them more susceptible to further neurodegenerative mechanisms.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Neurologi (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Neurology (hsv//eng)

Keyword

amyotrophic lateral sclerosis
metabolism
mitochondria

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art (subject category)

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