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lüll TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration Lagier-Tourenne C; Polymenidou M; Cleveland DWHum Mol Genet 2010[Apr]; 19 (R1): R46-64Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are neurodegenerative diseases with clinical and pathological overlap. Landmark discoveries of mutations in the transactive response DNA-binding protein (TDP-43) and fused in sarcoma/translocated in liposarcoma (FUS/TLS) as causative of ALS and FTLD, combined with the abnormal aggregation of these proteins, have initiated a shifting paradigm for the underlying pathogenesis of multiple neurodegenerative diseases. TDP-43 and FUS/TLS are both RNA/DNA-binding proteins with striking structural and functional similarities. Their association with ALS and other neurodegenerative diseases is redirecting research efforts toward understanding the role of RNA processing regulation in neurodegeneration.|Amyotrophic Lateral Sclerosis/genetics/*pathology[MESH]|Biological Transport[MESH]|DNA-Binding Proteins/metabolism/*physiology[MESH]|Frontotemporal Lobar Degeneration/genetics/*pathology[MESH]|Humans[MESH]|RNA Processing, Post-Transcriptional/*physiology[MESH]|RNA-Binding Protein FUS/metabolism/*physiology[MESH]|Subcellular Fractions/metabolism[MESH] |