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Article

Mitochondrial Dysfunction in Sporadic Amyotrophic Lateral Sclerosis Patients: Insights from High-Resolution Respirometry

by
Petra Parvanovova
1,
Andrea Evinova
2,
Milan Grofik
3,
Petra Hnilicova
2,
Zuzana Tatarkova
1 and
Monika Turcanova-Koprusakova
3,*
1
Department of Medical Biochemistry, Jessenius Faculty of Medicine, Comenius University in Bratislava, 036 01 Martin, Slovakia
2
Biomedical Centre Martin, Jessenius Faculty of Medicine, Comenius University in Bratislava, 036 01 Martin, Slovakia
3
Department of Neurology, University Hospital Martin, 036 01 Martin, Slovakia
*
Author to whom correspondence should be addressed.
Biomedicines 2024, 12(6), 1294; https://doi.org/10.3390/biomedicines12061294
Submission received: 13 May 2024 / Revised: 4 June 2024 / Accepted: 6 June 2024 / Published: 11 June 2024

Abstract

Amyotrophic lateral sclerosis is a severe neurodegenerative disease whose exact cause is still unclear. Currently, research attention is turning to the mitochondrion as a critical organelle of energy metabolism. Current knowledge is sufficient to confirm the involvement of the mitochondria in the pathophysiology of the disease, since the mitochondria are involved in many processes in the cell; however, the exact mechanism of involvement is still unclear. We used peripheral blood mononuclear cells isolated from whole fresh blood from patients with amyotrophic lateral sclerosis for measurement and matched an age- and sex-matched set of healthy subjects. The group of patients consisted of patients examined and diagnosed at the neurological clinic of the University Hospital Martin. The set of controls consisted of healthy individuals who were actively searched, and controls were selected on the basis of age and sex. The group consisted of 26 patients with sporadic forms of ALS (13 women, 13 men), diagnosed based on the definitive criteria of El Escorial. The average age of patients was 54 years, and the average age of healthy controls was 56 years. We used a high-resolution O2K respirometry method, Oxygraph-2k, to measure mitochondrial respiration. Basal respiration was lower in patients by 29.48%, pyruvate-stimulated respiration (respiratory chain complex I) was lower by 29.26%, and maximal respiratory capacity was lower by 28.15%. The decrease in succinate-stimulated respiration (respiratory chain complex II) was 26.91%. Our data confirm changes in mitochondrial respiration in ALS patients, manifested by the reduced function of complex I and complex II of the respiratory chain. These defects are severe enough to confirm this disease’s hypothesized mitochondrial damage. Therefore, research interest in the future should be directed towards a deeper understanding of the involvement of mitochondria and respiratory complexes in the pathophysiology of the disease. This understanding could develop new biomarkers in diagnostics and subsequent therapeutic interventions.
Keywords: amyotrophic lateral sclerosis; mitochondria; high-resolution respirometry; mitochondrion dysfunction; complex I; complex II amyotrophic lateral sclerosis; mitochondria; high-resolution respirometry; mitochondrion dysfunction; complex I; complex II

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MDPI and ACS Style

Parvanovova, P.; Evinova, A.; Grofik, M.; Hnilicova, P.; Tatarkova, Z.; Turcanova-Koprusakova, M. Mitochondrial Dysfunction in Sporadic Amyotrophic Lateral Sclerosis Patients: Insights from High-Resolution Respirometry. Biomedicines 2024, 12, 1294. https://doi.org/10.3390/biomedicines12061294

AMA Style

Parvanovova P, Evinova A, Grofik M, Hnilicova P, Tatarkova Z, Turcanova-Koprusakova M. Mitochondrial Dysfunction in Sporadic Amyotrophic Lateral Sclerosis Patients: Insights from High-Resolution Respirometry. Biomedicines. 2024; 12(6):1294. https://doi.org/10.3390/biomedicines12061294

Chicago/Turabian Style

Parvanovova, Petra, Andrea Evinova, Milan Grofik, Petra Hnilicova, Zuzana Tatarkova, and Monika Turcanova-Koprusakova. 2024. "Mitochondrial Dysfunction in Sporadic Amyotrophic Lateral Sclerosis Patients: Insights from High-Resolution Respirometry" Biomedicines 12, no. 6: 1294. https://doi.org/10.3390/biomedicines12061294

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