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 ActivityEvoked Potentials, MotorMutationSpinal Nerve RootsToxascariasisbeta-Hexosaminidase beta ChainNeuromuscular JunctionRNA-Binding Protein FUSRiluzoleUlnar NerveMuscle, SkeletalPharyngostomyPyrrolidonecarboxylic AcidBrainElectric InjuriesDeglutition DisordersPick Disease of the BrainPeripheral NervesBrain Injury, ChronicAction PotentialsLathyrismMuscle WeaknessPedigreeRibonucleoproteins, Small NuclearNeurologic ExaminationPhenotypeSandhoff DiseaseRats, TransgenicMarchiafava-Bignami DiseaseParalysisReceptors, AndrogenMusclesDementiaAxonal TransportAge of OnsetElectric StimulationCricoid CartilageOptic AtrophyImmunohistochemistryDNA Repeat ExpansionCells, CulturedTime FactorsRecruitment, NeurophysiologicalAnimals, Genetically ModifiedSpinal NervesMice, Inbred C57BLExonsCoiled BodiesMagnetic Resonance ImagingInjections, SpinalGanglia, SpinalChromosomes, Human, Pair 5Mutation, MissenseNervous System DiseasesSpastic Paraplegia, HereditarySynapsesMice, KnockoutDisease ProgressionNeuroprotective AgentsGastrostomy