title: RNA-binding proteins and RNA metabolism: a new scenario in the pathogenesis of Amyotrophic lateral sclerosis.
abstract: Several RNA-processing genes have been implicated in the pathogenesis of Amyotrophic lateral sclerosis (ALS). In particular, causative mutations in the genes encoding for two DNA/RNA binding proteins, TAR DNA binding protein-43 (TDP-43) and fused in sarcoma/translocated in liposarcoma (FUS/TLS), were recently identified in ALS patients. These genetic findings and the presence of abnormal aggregates of these two RNA-binding proteins in ALS affected tissues suggest that molecular mechanisms regulating RNA metabolism are implicated in ALS pathogenesis through common pathways. In this review similarities and differences between TDP-43 and FUS/TLS proteins and their activities in physiological and pathological conditions will be discussed.
authors.affiliation: Department of Neurology and Laboratory of Neuroscience IRCCS Istituto Auxologico Italiano, 20149 Milan, Italy. claudiacolombrita@hotmail.com
authors.forename: Claudia
authors.initials: C
authors.lastname: Colombrita
authors.forename: Elisa
authors.initials: E
authors.lastname: Onesto
authors.forename: Cinzia
authors.initials: C
authors.lastname: Tiloca
authors.forename: Nicola
authors.initials: N
authors.lastname: Ticozzi
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Silani
authors.forename: Antonia
authors.initials: A
authors.lastname: Ratti
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics* / Q000473:pathology*; D004268:DNA-Binding Proteins / Q000235:genetics; D006801:Humans; D008954:Models, Biological; D012313:RNA / Q000378:metabolism*; D034702:RNA-Binding Protein FUS / Q000235:genetics; D016601:RNA-Binding Proteins / Q000235:genetics* / Q000378:metabolism*
chemical_list: D004268:DNA-Binding Proteins; D034702:RNA-Binding Protein FUS; D016601:RNA-Binding Proteins; D012313:RNA