The DOI matches its Crossref record; 26 of the 27 references it cites were found in a registry or an index.
Impairment of mitochondrial function in skeletal muscle of patients with amyotrophic lateral sclerosis
Abstract
In skeletal muscle homogenates of 14 patients with sporadic amyotrophic lateral sclerosis, an approximately twofold lower specific activity of NADH:CoQ oxidoreductase in comparison to an age matched control group (n=28) was detected. This finding was confirmed by a detailed analysis of mitochondrial oxidative phosphorylation in skeletal muscle using saponin-permeabilized muscle fibers. (i) A significantly lowered maximal glutamate+malate and pyruvate+malate supported respiration of saponin-permeabilized fibers was detected in the patients group. (ii) Titrations with the specific inhibitor of NADH:CoQ oxidoreductase amytal revealed a higher sensitivity of respiration to this inhibitor indicating an elevated flux control coefficient of this enzyme. (iii) Applying functional imaging of mitochondria using ratios of NAD(P)H and flavoprotein autofluorescence images of saponin-permeabilized fibers we detected the presence of partially respiratory chain inhibited mitochondria on the single fiber level. A secondary defect of mitochondrial function due to the neurogenic changes in muscle seems to be unlikely since no mitochondrial abnormalities were detectable in biopsies of patients with spinal muscular atrophy. These results support the viewpoint that an impairment of mitochondria may be of pathophysiological significance in the etiology of amyotrophic lateral sclerosis.
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“Accumulations of dysmorphic and enlarged mitochondria, many with deficiencies of critical oxidative enzymes, in anterior horn cells, intramuscular nerves, and skeletal muscle are characteristic of sALS [281–285].”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [285]
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Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation
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Molecular biology of amyotrophic lateral sclerosis: insights from genetics
Pasinelli, P., & Brown, R. H. (2006) · Nature Reviews Neuroscience · doi:10.1038/nrn1971 · Cited by 1,107
Wijesekera, L. C., & Leigh, P. N. (2009) · Orphanet Journal of Rare Diseases · doi:10.1186/1750-1172-4-3 · Cited by 1,130
Statin Adverse Effects: A Review of the Literature and Evidence for a Mitochondrial Mechanism
Golomb, B. A., & Evans, M. A. (2008) · American Journal of Cardiovascular Drugs · doi:10.2165/0129784-200808060-00004 · Cited by 677
The role of mitochondria in amyotrophic lateral sclerosis
Smith, E. F., Shaw, P. J., & Vos, K. J. D. (2017) · Neuroscience Letters · doi:10.1016/j.neulet.2017.06.052 · Cited by 579
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Metadata
- Authors
- Wiedemann, F. R., Winkler, K., Kuznetsov, A. V., Bartels, C., Vielhaber, S., Feistner, H., & Kunz, W. S.
- Affiliations
- Klinikum Magdeburg (DE)
- University Hospital Magdeburg (DE)
- Corresponding author
- Kunz, Wolfram S
- Venue
- Journal of the Neurological Sciences
- Publisher
- Elsevier BV
- Published
- 1998-03
- Volume
- 156
- Issue
- 1
- Pages
- 65-72
- ISSN
- 0022-510X (ISSN-L)
- OpenAlex
- W2020074935
- Cited by
- 262
- References
- 27
- Citations by year
- 2026: 9 · 2025: 7 · 2024: 7 · 2023: 5 · 2022: 10 · 2021: 9 · 2020: 9 · 2019: 6 · 2018: 6 · 2017: 8 · 2016: 10 · 2015: 9 · 2014: 10 · 2013: 11 · 2012: 14
- FWCI
- 6.41
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of the Neurological Sciences (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Coenzyme Q10 studies and effects
- Keywords
- amyotrophic lateral sclerosis, mitochondrial dysfunction, skeletal muscle, oxidative phosphorylation, electron transport inhibition, amytal, flux control coefficients, NAD(P)H autofluorescence, spinal muscular atrophy
- MeSH terms
- Adult, Aged, Amyotrophic Lateral Sclerosis, Electromyography, Electron Transport, Female, Humans, Male, Microscopy, Fluorescence, Middle Aged, Mitochondria, Muscle, NAD(P)H Dehydrogenase (Quinone), Muscle, Skeletal
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