title: Mitochondrial alterations in fanconi anemia fibroblasts following ultraviolet A or psoralen photoactivation.
abstract: The genetic disease Fanconi anemia (FA), generally considered to be a DNA repair defect, has also been related to a deficiency in cellular defense against reactive oxygen species (ROS). Results show that mitochondrial matrix densification occurs rapidly and transiently in FA fibroblasts following 8-methoxypsoralen (8-MOP) photoreaction or ultraviolet A (320 to 380 nm) (UVA) irradiation. This effect is oxygen dependent because it is more important under 20 than under 5% oxygen tension. In contrast, in normal fibroblasts very little, if any, densification of mitochondrial matrix is induced by treatments even at the highest oxygen tension. The changes in matrix density in FA cells are accompanied by some modifications in transmembrane potential, linked to a Fenton-like reaction, and in mitochondrial cardiolipin content, differing from the responses of normal cells. These data are indicative of some sort of membrane damage induced by 8-MOP photoreaction and UVA irradiation, to which FA cells appear to be particularly sensitive.
authors.affiliation: UMR 218 CNRS, Institut Curie-Recherche, Paris, France. solange.rousset@curie.u-psud.fr
authors.forename: Solange
authors.initials: S
authors.lastname: Rousset
authors.forename: Silvano
authors.initials: S
authors.lastname: Nocentini
authors.forename: Danièlie
authors.initials: D
authors.lastname: Rouillard
authors.forename: Christiane
authors.initials: C
authors.lastname: Baroche
authors.forename: Ethel
authors.initials: E
authors.lastname: Moustacchi
mesh_terms: D002460:Cell Line; D005199:Fanconi Anemia / Q000473:pathology*; D005347:Fibroblasts / Q000473:pathology / Q000528:radiation effects* / Q000648:ultrastructure; D011564:Furocoumarins / Q000494:pharmacology; D006801:Humans; D008730:Methoxsalen / Q000494:pharmacology; D008928:Mitochondria / Q000473:pathology / Q000528:radiation effects*; D010100:Oxygen / Q000494:pharmacology; D017319:Photosensitizing Agents / Q000494:pharmacology*; D014466:Ultraviolet Rays
chemical_list: D011564:Furocoumarins; D017319:Photosensitizing Agents; D010100:Oxygen; D008730:Methoxsalen