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Mitochondria in Multiple Sclerosis: Molecular Mechanisms of Pathogenesis

2016; Elsevier BV; Linguagem: Inglês

10.1016/bs.ircmb.2016.08.003

1937-6448

Simone Patergnani, Valentina Fossati, Massimo Bonora, Carlotta Giorgi, Saverio Marchi, Sonia Missiroli, Tom Rusielewicz, Mariusz R. Wiȩckowski, Paolo Pinton,

RNA Research and Splicing

Mitochondria, the organelles that function as the powerhouse of the cell, have been increasingly linked to the pathogenesis of many neurological disorders, including multiple sclerosis (MS). MS is a chronic inflammatory demyelinating disease of the central nervous system (CNS) and a leading cause of neurological disability in young adults in the western world. Its etiology remains unknown, and while the inflammatory component of MS has been heavily investigated and targeted for therapeutic intervention, the failure of remyelination and the process of axonal degeneration are still poorly understood. Recent studies suggest a role of mitochondrial dysfunction in the neurodegenerative aspects of MS. This review is focused on mitochondrial functions under physiological conditions and the consequences of mitochondrial alterations in various CNS disorders. Moreover, we summarize recent findings linking mitochondrial dysfunction to MS and discuss novel therapeutic strategies targeting mitochondria-related pathways as well as emerging experimental approaches for modeling mitochondrial disease.