Mitochondria in Sporadic Amyotrophic Lateral Sclerosis

• Published in: Experimental neurology Vol. 153; no. 1; pp. 135 - 142
• Main Authors: Swerdlow, Russell H., Parks, Janice K., Cassarino, David S., Trimmer, Patricia A., Miller, Scott W., Maguire, David J., Sheehan, Jason P., Maguire, Robyn S., Pattee, Gary, Juel, Vern C., Phillips, Lawrence H., Tuttle, Jeremy B., Bennett, James P., Davis, Robert E., Parker, W.Davis
• Format: Journal Article
• Language: English
• Published: Amsterdam Elsevier Inc 01.09.1998; Elsevier
• Subjects: 1-Methyl-4-phenylpyridinium - metabolism; ALS; amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - genetics; Amyotrophic Lateral Sclerosis - pathology; Animals; Biological and medical sciences; calcium; Calcium - metabolism; Cell Line; cybrids; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; DNA; DNA, Mitochondrial - genetics; DNA, Mitochondrial - metabolism; Electron Transport - genetics; Electron Transport - physiology; Female; Free Radical Scavengers - metabolism; Humans; Hybrid Cells; Male; Medical sciences; Mice; Microscopy, Electron; Middle Aged; mitochondria; Mitochondria - metabolism; Mitochondria - physiology; Mitochondria - ultrastructure; Mutation - genetics; Neurology; oxidative; calcium; DNA; mitochondria; ALS; oxidative; amyotrophic lateral sclerosis; cybrids; Human; Nervous system diseases; Sporadic; Pathogenesis; Central nervous system disease; Amyotrophic lateral sclerosis; Degenerative disease; Mitochondrial DNA; Mutation; Spinal cord disease; Mitochondrial disorder

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...