The DOI matches its Crossref record; 5 of the 5 references it cites were found in a registry or an index.
Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients
Abstract
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by selective motor neuron death. In order to address the question of a putative role of mitochondrial dysfunction in the pathogenesis of ALS, we studied the mitochondrial DNA (mtDNA) and mitochondrial respiratory chain enzyme activities in spinal cords of ALS patients and in control subjects without neuropathologic abnormalities. Using a ‘double PCR and digestion’ technique to estimate the levels of randomly distributed point mutations in two small regions of the mtDNA, we found significantly higher levels of mutant mtDNA in the spinal cord of ALS patients compared to controls. No large‐scale rearrangements were found, but the amount of mtDNA, measured by Southern blot, was significantly lower in the ALS samples. This reduction correlated well with a decrease of citrate synthase (CS) activity, a mitochondrial marker, as were the activities of respiratory chain complexes I + III, II + III, and IV, suggesting a loss of mitochondria in ALS spinal cords.
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Rosen, D. R., Siddique, T., Patterson, D., Figlewicz, D. A., Sapp, P., Hentati, A., Donaldson, D., Goto, J., O'Regan, J. P., Deng, H.-X., et al. (1993) · Nature · doi:10.1038/362059a0 · Cited by 7,062
Goto, Y.-I., Nonaka, I., & Horai, S. (1990) · Nature · doi:10.1038/348651a0 · Cited by 2,070
Skulachev, V. P. (1996) · Quarterly Reviews of Biophysics · doi:10.1017/s0033583500005795 · Cited by 703
Wiedemann, F. R., Winkler, K., Kuznetsov, A. V., Bartels, C., Vielhaber, S., Feistner, H., & Kunz, W. S. (1998) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(98)00008-2 · Cited by 262
Self-citationSasaki, S., & Iwata, M. (1996) · Neuroscience Letters · doi:10.1016/0304-3940(96)12314-4 · Cited by 173
Cited by
Kiernan, M. C., Vucic, S., Cheah, B. C., Turner, M. R., Eisen, A., Hardiman, O., Burrell, J. R., & Zoing, M. C. (2011) · The Lancet · doi:10.1016/s0140-6736(10)61156-7 · Cited by 2,603
ALS: A Disease of Motor Neurons and Their Nonneuronal Neighbors
Boillée, S., Vande Velde, C., & Cleveland, D. W. (2006) · Neuron · doi:10.1016/j.neuron.2006.09.018 · Cited by 1,429
Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis
Ferraiuolo, L., Kirby, J., Grierson, A. J., Sendtner, M., & Shaw, P. J. (2011) · Nature Reviews Neurology · doi:10.1038/nrneurol.2011.152 · Cited by 615
The role of mitochondria in amyotrophic lateral sclerosis
Smith, E. F., Shaw, P. J., & De Vos, K. J. (2019) · Neuroscience Letters · doi:10.1016/j.neulet.2017.06.052 · Cited by 559
Oxidative stress in ALS: A mechanism of neurodegeneration and a therapeutic target
Barber, S. C., Mead, R. J., & Shaw, P. J. (2006) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2006.03.008 · Cited by 474
Metadata
- Authors
- Wiedemann, F. R., Manfredi, G., Mawrin, C., Beal, M. F., & Schon, E. A.
- Venue
- Journal of Neurochemistry
- Publisher
- Wiley
- Published
- 2002-02
- Volume
- 80
- Issue
- 4
- Pages
- 616-625
- ISSN
- 0022-3042 (ISSN-L)
- 1471-4159
- OpenAlex
- W2038697027
- Cited by
- 359
- References
- 5
- FWCI
- 8.16
- Type
- journal article
- Language
- en
- Open access
- closed
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- Wiedemann, F. R., Manfredi, G., Mawrin, C., Beal, M. F., & Schon, E. A. (2002). Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients. Journal of Neurochemistry. https://doi.org/10.1046/j.0022-3042.2001.00731.x
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
