Amyotrophic Lateral SclerosisRNA-Binding Protein FUSSuperoxide DismutasePoint MutationMutation, MissenseMutationMotor Neuron DiseaseMotor NeuronsFrameshift MutationGerm-Line MutationPedigreeSpinal CordAmino Acid SubstitutionDNA Repeat ExpansionMice, TransgenicExonsMutation RateFrontotemporal DementiaHeterozygoteBase SequenceRiluzoleInclusion BodiesMolecular Sequence DataDisease Models, AnimalGuamDNA Mutational AnalysisPolymerase Chain ReactionFrontotemporal Lobar DegenerationAllelesAmino Acid SequenceDNA-Binding ProteinsNerve DegenerationGenotypeMutagenesis, Site-DirectedMutant ProteinsHomozygotePhenotypePolymorphism, Single-Stranded ConformationalNeurodegenerative DiseasesSequence Analysis, DNACodon, NonsenseMutagenesisGenes, DominantProtein Structure, TertiaryGenes, RecessiveAge of OnsetCell LineAnterior Horn CellsFasciculationGenetic TestingModels, MolecularSequence DeletionTDP-43 ProteinopathiesDNA PrimersGenetic Predisposition to DiseaseChromosome MappingDisease ProgressionProtein BindingBinding SitesBulbar Palsy, ProgressiveEscherichia coliNeurofilament ProteinsSuppression, GeneticOxidative StressGene DeletionFamily HealthSyndromeRats, TransgenicMitochondriaDNATransfectionCodonBrainGenetic LinkageSequence Homology, Amino AcidTranscription FactorsProtein Folding