Apoptotic Cell Death of Cybrid Cells Bearing Leber's Hereditary Optic Neuropathy Mutations Is Caspase Independent
- 1 December 2003
- journal article
- website
- Published by Wiley in Annals of the New York Academy of Sciences
- Vol. 1010 (1) , 213-217
- https://doi.org/10.1196/annals.1299.037
Abstract
Abstract: Leber's hereditary optic neuropathy (LHON) is a maternally inherited disease characterized by selective death of retinal ganglion cells. Three pathogenic mtDNA point mutations induce an impairment of oxidative phosphorylation. We have investigated whether the release of cytochrome c during incubation of LHON cybrids in galactose medium leads to activation of the executive caspase‐3 and to alteration of the energetic status of cells. From our research, it can be concluded that apoptotic cell death induced in LHON cybrid by galactose medium is caspase independent. It remains to be explained how the significant fragmentation of intranucleosomal DNA observed in LHON cybrids could also occur in the absence of caspase activation.Keywords
This publication has 5 references indexed in Scilit:
- Leber's Hereditary Optic Neuropathy (LHON) Pathogenic Mutations Induce Mitochondrial-dependent Apoptotic Death in Transmitochondrial Cells Incubated with Galactose MediumJournal of Biological Chemistry, 2003
- 7-Ketocholesterol and staurosporine induce opposite changes in intracellular pH, associated with distinct types of cell death in ECV304 cellsArchives of Biochemistry and Biophysics, 2002
- Cells Bearing Mutations Causing Leber's Hereditary Optic Neuropathy Are Sensitized to Fas-induced ApoptosisJournal of Biological Chemistry, 2002
- Functional Analysis of Lymphoblast and Cybrid Mitochondria Containing the 3460, 11778, or 14484 Leber's Hereditary Optic Neuropathy Mitochondrial DNA MutationJournal of Biological Chemistry, 2000
- Induction of Apoptotic Program in Cell-Free Extracts: Requirement for dATP and Cytochrome cPublished by Elsevier ,1996