title: Apoptotic cell death of cybrid cells bearing Leber's hereditary optic neuropathy mutations is caspase independent.
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.
authors.affiliation: Dipartimento di Biologia Evoluzionistica Sperimentale, Universita di Bologna, Bologna, Italy. zanna@alma.unibo.it
authors.forename: Claudia
authors.initials: C
authors.lastname: Zanna
authors.forename: Anna
authors.initials: A
authors.lastname: Ghelli
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Porcelli
authors.forename: Valerio
authors.initials: V
authors.lastname: Carelli
authors.forename: Andrea
authors.initials: A
authors.lastname: Martinuzzi
authors.forename: Michela
authors.initials: M
authors.lastname: Rugolo
mesh_terms: D000255:Adenosine Triphosphate / Q000378:metabolism; D017209:Apoptosis / Q000235:genetics*; D020169:Caspases / Q000378:metabolism*; D002478:Cells, Cultured; D045304:Cytochromes c / Q000032:analysis; D004272:DNA, Mitochondrial / Q000235:genetics; D006801:Humans; D008928:Mitochondria / Q000502:physiology / Q000637:transplantation / Q000648:ultrastructure; D029242:Optic Atrophy, Hereditary, Leber / Q000235:genetics* / Q000473:pathology; D017354:Point Mutation
chemical_list: D004272:DNA, Mitochondrial; D000255:Adenosine Triphosphate; D045304:Cytochromes c; D020169:Caspases