title: Krabbe's leukodystrophy: Approaches and models in vitro.
abstract: This Review describes some in vitro approaches used to investigate the mechanisms involved in Krabbe's disease, with particular regard to the cellular systems employed to study processes of inflammation, apoptosis, and angiogenesis. The aim was to update the knowledge on the results obtained from in vitro models of this neurodegenerative disorder and provide stimuli for future research. For a long time, the nonavailability of established neural cells has limited the understanding of neuropathogenic mechanisms in Krabbe's leukodystrophy. More recently, the development of new Krabbe's disease cell models has allowed the identification of neurologically relevant pathogenic cascades, including the major role of elevated psychosine levels. Thus, direct and/or indirect roles of psychosine in the release of cytokines, reactive oxygen species, and nitric oxide and in the activation of kinases, caspases, and angiogenic factors results should be clearer. In parallel, it is now understood that the presence of globoid cells precedes oligodendrocyte apoptosis and demyelination. The information described here will help to continue the research on Krabbe's leukodystrophy and on potential new therapeutic approaches for this disease that even today, despite numerous attempts, is without cure. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Department of Biomedical and Biotechnological Sciences, Section of Physiology, University of Catania, Catania, Italy.
authors.forename: Rosanna
authors.initials: R
authors.lastname: Avola
authors.affiliation: Department of Biomedical and Biotechnological Sciences, Section of Physiology, University of Catania, Catania, Italy.
authors.forename: Adriana Carol Eleonora
authors.initials: AC
authors.lastname: Graziano
authors.affiliation: Department of Biomedical and Biotechnological Sciences, Section of Physiology, University of Catania, Catania, Italy.
authors.forename: Giovanna
authors.initials: G
authors.lastname: Pannuzzo
authors.affiliation: Department of Biomedical and Biotechnological Sciences, Section of Physiology, University of Catania, Catania, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Alvares
authors.affiliation: Department of Biomedical and Biotechnological Sciences, Section of Physiology, University of Catania, Catania, Italy. cardile@unict.it.
authors.forename: Venera
authors.initials: V
authors.lastname: Cardile
mesh_terms: D000818:Animals; D017209:Apoptosis / Q000502:physiology; D001921:Brain / Q000378:metabolism*; D016207:Cytokines / Q000378:metabolism; D006801:Humans; D066298:In Vitro Techniques; D007249:Inflammation / Q000209:etiology; D007965:Leukodystrophy, Globoid Cell / Q000150:complications / Q000378:metabolism* / Q000473:pathology*; D009389:Neovascularization, Pathologic / Q000209:etiology; D011609:Psychosine / Q000378:metabolism*; D017382:Reactive Oxygen Species
chemical_list: D016207:Cytokines; D017382:Reactive Oxygen Species; D011609:Psychosine
keywords: angiogenesis; apoptosis; cell models; globoid cell leukodystrophy; in vitro experiment; inflammation