Mitochondria in sporadic amyotrophic lateral sclerosis.
Publication
, Journal Article
Swerdlow, RH; Parks, JK; Cassarino, DS; Trimmer, PA; Miller, SW; Maguire, DJ; Sheehan, JP; Maguire, RS; Pattee, G; Juel, VC; Phillips, LH ...
Published in: Exp Neurol
September 1998
Mitochondria are abnormal in persons with amyotrophic lateral sclerosis (ALS) for unknown reasons. We explored whether aberration of mitochondrial DNA (mtDNA) could play a role in this by transferring mitochondrial DNA (mtDNA) from ALS subjects to mtDNA-depleted human neuroblastoma cells. Resulting ALS cytoplasmic hybrids (cybrids) exhibited abnormal electron transport chain functioning, increases in free radical scavenging enzyme activities, perturbed calcium homeostasis, and altered mitochondrial ultrastructure. Recapitulation of defects previously observed in ALS subjects and ALS transgenic mice by expression of ALS mtDNA support a pathophysiologic role for mtDNA mutation in some persons with this disease.
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Published In
Exp Neurol
DOI
ISSN
0014-4886
Publication Date
September 1998
Volume
153
Issue
1
Start / End Page
135 / 142
Location
United States
Related Subject Headings
- Neurology & Neurosurgery
- Mutation
- Mitochondria
- Middle Aged
- Microscopy, Electron
- Mice
- Male
- Hybrid Cells
- Humans
- Free Radical Scavengers
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Swerdlow, R. H., Parks, J. K., Cassarino, D. S., Trimmer, P. A., Miller, S. W., Maguire, D. J., … Parker, W. D. (1998). Mitochondria in sporadic amyotrophic lateral sclerosis. Exp Neurol, 153(1), 135–142. https://doi.org/10.1006/exnr.1998.6866
Swerdlow, R. H., J. K. Parks, D. S. Cassarino, P. A. Trimmer, S. W. Miller, D. J. Maguire, J. P. Sheehan, et al. “Mitochondria in sporadic amyotrophic lateral sclerosis.” Exp Neurol 153, no. 1 (September 1998): 135–42. https://doi.org/10.1006/exnr.1998.6866.
Swerdlow RH, Parks JK, Cassarino DS, Trimmer PA, Miller SW, Maguire DJ, et al. Mitochondria in sporadic amyotrophic lateral sclerosis. Exp Neurol. 1998 Sep;153(1):135–42.
Swerdlow, R. H., et al. “Mitochondria in sporadic amyotrophic lateral sclerosis.” Exp Neurol, vol. 153, no. 1, Sept. 1998, pp. 135–42. Pubmed, doi:10.1006/exnr.1998.6866.
Swerdlow RH, Parks JK, Cassarino DS, Trimmer PA, Miller SW, Maguire DJ, Sheehan JP, Maguire RS, Pattee G, Juel VC, Phillips LH, Tuttle JB, Bennett JP, Davis RE, Parker WD. Mitochondria in sporadic amyotrophic lateral sclerosis. Exp Neurol. 1998 Sep;153(1):135–142.
Published In
Exp Neurol
DOI
ISSN
0014-4886
Publication Date
September 1998
Volume
153
Issue
1
Start / End Page
135 / 142
Location
United States
Related Subject Headings
- Neurology & Neurosurgery
- Mutation
- Mitochondria
- Middle Aged
- Microscopy, Electron
- Mice
- Male
- Hybrid Cells
- Humans
- Free Radical Scavengers