Motor Neuron DiseaseMuscular Atrophy, SpinalMotor NeuronsNeuromuscular DiseasesSpinal Muscular Atrophies of ChildhoodBulbar Palsy, ProgressiveSurvival of Motor Neuron 1 ProteinSMN Complex ProteinsSpinal CordSurvival of Motor Neuron 2 ProteinAmyotrophic Lateral SclerosisMuscular AtrophyAtrophyNeuronsMuscular Disorders, AtrophicBulbo-Spinal Atrophy, X-LinkedAnterior Horn CellsNerve DegenerationNerve Tissue ProteinsSpinal Cord InjuriesAxonsSpinal Cord DiseasesFasciculationSuperoxide DismutaseNeuronal Apoptosis-Inhibitory ProteinDEAD Box Protein 20Cyclic AMP Response Element-Binding ProteinElectromyographyRNA-Binding ProteinsDisease Models, AnimalMotor CortexNeurons, AfferentNeurofilament ProteinsGaggingNeural ConductionMice, TransgenicTDP-43 ProteinopathiesInclusion BodiesFrontotemporal Lobar DegenerationFrontotemporal DementiaMotor ActivityMutationEvoked Potentials, MotorSpinal Nerve RootsToxascariasisbeta-Hexosaminidase beta ChainNeuromuscular JunctionRNA-Binding Protein FUSMuscle, SkeletalRiluzoleUlnar NervePharyngostomyPyrrolidonecarboxylic AcidBrainElectric InjuriesDeglutition DisordersPick Disease of the BrainPeripheral NervesBrain Injury, ChronicAction PotentialsLathyrismMuscle WeaknessPedigreeNeurologic ExaminationRibonucleoproteins, Small NuclearPhenotypeSandhoff DiseaseRats, TransgenicParalysisMarchiafava-Bignami DiseaseReceptors, AndrogenMusclesDementiaAge of OnsetAxonal TransportElectric StimulationImmunohistochemistryCricoid CartilageOptic AtrophyTime FactorsDNA Repeat ExpansionCells, CulturedRecruitment, NeurophysiologicalAnimals, Genetically ModifiedSpinal NervesMice, Inbred C57BLExonsMagnetic Resonance ImagingCoiled BodiesInjections, SpinalGanglia, SpinalChromosomes, Human, Pair 5Mutation, MissenseNervous System DiseasesSynapsesSpastic Paraplegia, HereditaryDisease ProgressionMice, KnockoutNeuroprotective AgentsGastrostomy