Devoy, Anny Humanized mutant FUS drives progressive motor neuron degeneration without aggregation in 'FUSDelta14' knockin mice. [electronic resource] - Brain : a journal of neurology Nov 2017 - 2797-2805 p. digital Publication Type: Journal Article ISSN: 1460-2156 Standard No.: 10.1093/brain/awx248 doi Subjects--Topical Terms: Amyotrophic Lateral Sclerosis--geneticsAnimalsDisease Models, AnimalEndoplasmic Reticulum, Rough--metabolismFrameshift MutationGene DosageGene Expression ProfilingGene Knock-In TechniquesHeterozygoteHumansMiceMitochondria--metabolismMotor Neurons--metabolismNeuromuscular Junction--metabolismProtein Aggregation, Pathological--geneticsRNA-Binding Protein FUS--geneticsRibosomal Proteins--genetics