Mitochondria in sporadic amyotrophic lateral sclerosis.

Journal Article (Journal Article)

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.

Full Text

Duke Authors

Cited Authors

  • 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

Published Date

  • September 1998

Published In

Volume / Issue

  • 153 / 1

Start / End Page

  • 135 - 142

PubMed ID

  • 9743575

International Standard Serial Number (ISSN)

  • 0014-4886

Digital Object Identifier (DOI)

  • 10.1006/exnr.1998.6866


  • eng

Conference Location

  • United States