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lüll RNA-binding proteins and RNA metabolism: a new scenario in the pathogenesis of Amyotrophic lateral sclerosis Colombrita C; Onesto E; Tiloca C; Ticozzi N; Silani V; Ratti AArch Ital Biol 2011[Mar]; 149 (1): 83-99Several 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.|Amyotrophic Lateral Sclerosis/*genetics/*pathology[MESH]|DNA-Binding Proteins/genetics[MESH]|Humans[MESH]|Models, Biological[MESH]|RNA-Binding Protein FUS/genetics[MESH]|RNA-Binding Proteins/*genetics/*metabolism[MESH]|RNA/*metabolism[MESH] |