title: Mitochondrial disfunction as a cause of ALS.
abstract: Recent studies on patient with sporadic ALS and on in vitro and in vivo models of mendelian diseases have been addressed toward the unravelling of the mitochondrial behaviour in ALS, whether as a primarily pathogenic factor, or as a fundamental contributor to the cell death. Morphological evidence suggests mitochondria pathology in ALS and many physiological mechanisms involving these organelles appear deranged in ALS, such as energy production, apoptotic triggering, calcium homeostasis and axonal transport of mitochondria. The article briefly addresses recent advances on this field.
authors.affiliation: Università di Roma "Sapienza" Dipartimento di Medicina Sperimentale, Istituto CSS-Mendel, Rome, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Pizzuti
authors.forename: Simona
authors.initials: S
authors.lastname: Petrucci
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000209:etiology* / Q000473:pathology; D000818:Animals; D001370:Axonal Transport / Q000502:physiology; D002118:Calcium / Q000378:metabolism; D004734:Energy Metabolism / Q000502:physiology; D006801:Humans; D008928:Mitochondria / Q000473:pathology* / Q000502:physiology / Q000648:ultrastructure; D028361:Mitochondrial Diseases / Q000150:complications*; D001678:Organelle Biogenesis
chemical_list: D002118:Calcium