ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS - López-Erauskin, Jone; Tadokoro, Takahiro; Baughn, Michael W.; Myers, Brian; McAlonis-Downes, Melissa; Chillon-Marinas, Carlos; Asiaban, Joshua N.; Artates, Jonathan; Bui, Anh T.; Vetto, Anne P.; Lee, Sandra K.; Le, Ai Vy; et al. (Neuron (Cambridge, Mass.), 2018)
Keywords: Age; ALS; Amyotrophic Lateral Sclerosis; Amyotrophic Lateral Sclerosis - genetics; Amyotrophic Lateral Sclerosis - metabolism; Amyotrophic Lateral Sclerosis - pathology; Animals; axonal translation; Axons - pathology; Axons - physiology; Behavior; Cells, Cultured; Chaperones; Cognitive ability; Dementia; Dementia disorders; Female; Frontotemporal dementia; Frontotemporal Dementia - genetics; Frontotemporal Dementia - metabolism; Frontotemporal Dementia - pathology; FTD; FUS; FUS protein; Gene expression; Hippocampus; Hippocampus - metabolism; Hippocampus - pathology; Humans; Ion channels; Localization; Loss of Function Mutation - genetics; Male; Medical research; Mice; Mice, Inbred C57BL; Mice, Transgenic; Mutation; Nerves; neurodegenerative disease; Neurons; Pregnancy; Protein biosynthesis; Protein Biosynthesis - physiology; Protein synthesis; Proteins; RNA-Binding Protein FUS - biosynthesis; RNA-Binding Protein FUS - genetics; RNA-binding proteins; Software; Spinal cord; Splicing; TLS; Toxicity; Washington DC; California; United Kingdom--UK; United States--US; Amyotrophic Lateral Sclerosis; neurodegenerative disease; FUS; FTD; ALS; protein synthesis; TLS; RNA-binding proteins; Frontotemporal dementia; axonal translation