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1. Full Text TDP-43 Proteinopathy and Motor Neuron Disease in Chronic Traumatic Encephalopathy
by McKee, Ann C and Gavett, Brandon E and Stern, Robert A and Nowinski, Christopher J and Cantu, Robert C and Kowall, Neil W and Perl, Daniel P and Hedley-Whyte, E. Tessa and Price, Bruce and Sullivan, Chris and Morin, Peter and Lee, Hyo-Soon and Kubilus, Caroline A and Daneshvar, Daniel H and Wulff, Megan and Budson, Andrew E
Journal of Neuropathology and Experimental Neurology, ISSN 0022-3069, 09/2010, Volume 69, Issue 9, pp. 918 - 929
Epidemiological evidence suggests that the incidence of amyotrophic lateral sclerosis is increased in association with head injury. Repetitive head injury is... 
Amyotrophic lateral sclerosis | Chronic brain injury | TDP-43 | Tau proteins | Motor neuron disease | Sports | HEAD-INJURY | ALZHEIMERS-DISEASE | AMYOTROPHIC-LATERAL-SCLEROSIS | PARKINSONISM-DEMENTIA COMPLEX | PATHOLOGY | FRONTOTEMPORAL LOBAR DEGENERATION | PROFESSIONAL FOOTBALL PLAYERS | GULF-WAR VETERANS | LOU-GEHRIGS-DISEASE | NEUROSCIENCES | CLINICAL NEUROLOGY | CIGARETTE-SMOKING | ANTECEDENT EVENTS | Motor Neuron Disease - etiology | Brain - anatomy & histology | Brain Injury, Chronic - pathology | Humans | Middle Aged | Brain Injury, Chronic - complications | Athletes | DNA-Binding Proteins - genetics | TDP-43 Proteinopathies - etiology | Motor Neuron Disease - pathology | Brain - metabolism | DNA-Binding Proteins - metabolism | Brain Injury, Chronic - physiopathology | Athletic Injuries - pathology | Aged, 80 and over | Athletic Injuries - complications | Brain - pathology | Adult | Aged | TDP-43 Proteinopathies - pathology | Motor Neuron Disease - physiopathology | TDP-43 Proteinopathies - physiopathology | Index Medicus
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2. Full Text Human endogenous retrovirus-K contributes to motor neuron disease
by Li, Wenxue and Lee, Myoung-Hwa and Henderson, Lisa and Tyagi, Richa and Bachani, Muzna and Steiner, Joseph and Campanac, Emilie and Hoffman, Dax A and Geldern, Gloria Von and Johnson, Kory and Maric, Dragan and Morris, H. Douglas and Lentz, Margaret and Pak, Katherine and Mammen, Andrew and Ostrow, Lyle and Rothstein, Jeffrey and Nath, Avindra
Science Translational Medicine, ISSN 1946-6234, 09/2015, Volume 7, Issue 307, pp. 307ra153 - 307ra153
The role of human endogenous retroviruses (HERVs) in disease pathogenesis is unclear. We show that HERV-K is activated in a subpopulation of patients with... 
MEDICINE, RESEARCH & EXPERIMENTAL | HUMAN TISSUES | PROTEIN | GENE | REVERSE-TRANSCRIPTASE ACTIVITY | ALS | DETERMINANT | SERUM | MICE | EXPRESSION | DEGENERATION | CELL BIOLOGY | Endogenous Retroviruses - physiology | Amyotrophic Lateral Sclerosis - physiopathology | Humans | Brain - virology | Amyotrophic Lateral Sclerosis - virology | Mice, Transgenic | Motor Neurons - pathology | Nerve Degeneration - pathology | Terminal Repeat Sequences - genetics | Motor Neuron Disease - pathology | Behavior, Animal | DNA-Binding Proteins - metabolism | Motor Neuron Disease - virology | Amyotrophic Lateral Sclerosis - pathology | Phenotype | Animals | Brain - pathology | Motor Neurons - virology | Motor Neuron Disease - physiopathology | Binding Sites | Virus Activation | Index Medicus
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3. Full Text Focal transplantation-based astrocyte replacement is neuroprotective in a model of motor neuron disease
by Dejea, Christine and Pardo, Andrea C and Rauck, Britta and Maragakis, Nicholas J and Rothstein, Jeffrey D and Lepore, Angelo C and Rao, Mahendra S
Nature Neuroscience, ISSN 1097-6256, 11/2008, Volume 11, Issue 11, pp. 1294 - 1301
Cellular abnormalities in amyotrophic lateral sclerosis (ALS) are not limited to motor neurons. Astrocyte dysfunction also occurs in human ALS and transgenic... 
GLIAL-RESTRICTED PRECURSORS | TRANSGENIC RAT MODEL | GLUTAMATE TRANSPORTERS | ADULT RATS | MOUSE MODEL | EXTEND SURVIVAL | ALS MODEL | AMYOTROPHIC-LATERAL-SCLEROSIS | HNT NEURONS | NEUROSCIENCES | STEM-CELL GRAFTS | Superoxide Dismutase - genetics | Phrenic Nerve - physiopathology | Humans | Glial Fibrillary Acidic Protein - metabolism | Cell Movement - physiology | Hindlimb - physiopathology | Motor Neuron Disease - therapy | Spinal Cord - pathology | Motor Neuron Disease - physiopathology | Cell Differentiation - physiology | Cell Survival - physiology | Disease Models, Animal | Calcium-Binding Proteins - metabolism | Neural Conduction - genetics | Animals, Genetically Modified | Excitatory Amino Acid Transporter 2 - deficiency | Rats | Cell Transplantation | Spinal Cord - surgery | Motor Neuron Disease - pathology | Action Potentials - physiology | Astrocytes - physiology | Animals | Green Fluorescent Proteins - biosynthesis | Microfilament Proteins | Muscle, Skeletal - physiopathology | Stem Cells - physiology | Spinal Cord - physiopathology | Physiological aspects | Motor neurons | Nervous system | Care and treatment | Degeneration | Astrocytes | Index Medicus | astroglia | grafting | neurodegeneration | ALS | neural precursor cell | progenitor | amyotrophic lateral sclerosis | SOD1 | non-cell autonomous | stem cell | motor neuron | neuroprotection | transplantation | lineage-restricted precursor | glial precursor | replacement | astrocyte
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4. Clinical neurophysiology of motor neuron diseases
by Eisen, Andrew, 1936
2004, 1st ed., Handbook of clinical neurophysiology, ISBN 0444513590, Volume 4, xv, 724
Amyotrophic lateral sclerosis | diagnosis | physiopathology | Pathophysiology | Motor Neuron Disease
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5. Full Text Motor Neuron dysfunction in frontotemporal dementia
by Burrell, James R and Kiernan, Matthew C and Vucic, Steve and Hodges, John R
Brain, ISSN 0006-8950, 9/2011, Volume 134, Issue 9, pp. 2582 - 2594
Frontotemporal dementia and motor neuron disease share clinical, genetic and pathological characteristics. Motor neuron disease develops in a proportion of... 
frontotemporal dementia | motor neuron disease | transcranial magnetic stimulation | biomarkers | BRAIN ATROPHY | LOBAR DEGENERATION | CORTICAL HYPEREXCITABILITY | THRESHOLD TRACKING | PRIMARY-PROGRESSIVE-APHASIA | ALS | AMYOTROPHIC-LATERAL-SCLEROSIS | NEUROSCIENCES | CLINICAL NEUROLOGY | DISEASE | CORTICOBASAL DEGENERATION | Biomarkers - metabolism | Frontotemporal Dementia - physiopathology | Motor Neurons - physiology | Cross-Sectional Studies | Humans | Middle Aged | Male | Motor Neurons - pathology | Motor Neuron Disease - pathology | Neuropsychological Tests | Transcranial Magnetic Stimulation | Aged, 80 and over | Aged | Motor Neuron Disease - physiopathology | Frontotemporal Dementia - pathology | Index Medicus | Abridged Index Medicus
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6. Full Text Traumatic brain injury results in cellular, structural and functional changes resembling motor neuron disease
by Wright, David K and Liu, Shijie and Van Der Poel, Chris and McDonald, Stuart J and Brady, Rhys D and Taylor, Lily and Yang, Li and Gardner, Andrew J and Ordidge, Roger and O'Brien, Terence J and Johnston, Leigh A and Shultz, Sandy R
Cerebral Cortex, ISSN 1047-3211, 2017, Volume 27, Issue 9, pp. 4503 - 4515
Traumatic brain injury (TBI) has been suggested to increase the risk of amyotrophic lateral sclerosis (ALS). However, this link remains controversial and as... 
Animal model | Amyotrophic lateral sclerosis | MRI | Neurodegeneration | TDP-43 | HEAD-INJURY | WHITE-MATTER INTEGRITY | neurodegeneration | AMYOTROPHIC-LATERAL-SCLEROSIS | amyotrophic lateral sclerosis | NEUROSCIENCES | VOXEL-BASED MORPHOMETRY | TENSOR-BASED MORPHOMETRY | DIFFUSION MRI | MUSCLE-FIBERS | CORTICOSPINAL TRACT | SPATIAL STATISTICS | animal model | TRANSGENIC MOUSE MODEL | DNA-Binding Proteins - metabolism | Motor Neuron Disease - etiology | Animals | Brain Injuries, Traumatic - complications | Amyotrophic Lateral Sclerosis - physiopathology | Rats, Long-Evans | Cytoplasm - metabolism | Male | Motor Cortex - physiopathology | Spinal Cord - physiopathology | Motor Neuron Disease - physiopathology | Disease Models, Animal | Index Medicus
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7. Full Text Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP
by Josephs, K.A and Petersen, R.C and Knopman, D.S and Boeve, B.F and Whitwell, J.L and Duffy, J.R and Parisi, J.E and Dickson, D.W
Neurology, ISSN 0028-3878, 01/2006, Volume 66, Issue 1, pp. 41 - 48
Objective: To examine the relationship between early clinical features, pathologies, and biochemistry of the frontotemporal lobar degenerations (FTLDs),... 
RICHARDSON-OLSZEWSKI-SYNDROME | DEMENTIA | LOBAR DEGENERATION | PICKS-DISEASE | NEUROPATHOLOGIC CRITERIA | MOTOR-NEURON DISEASE | CLINICAL NEUROLOGY | PROGRESSIVE SUPRANUCLEAR PALSY | PATHOLOGICAL DIAGNOSIS | NONFLUENT APHASIA | TAU-PATHOLOGY | Immunohistochemistry | Neurons - pathology | Predictive Value of Tests | Dementia - classification | Supranuclear Palsy, Progressive - physiopathology | Humans | Middle Aged | tau Proteins - metabolism | Dementia - diagnosis | Dementia - physiopathology | Male | Aphasia, Primary Progressive - diagnosis | Female | Neurons - metabolism | Inclusion Bodies - metabolism | Motor Neuron Disease - physiopathology | Aphasia, Primary Progressive - physiopathology | Tauopathies - physiopathology | Biomarkers - metabolism | Diagnosis, Differential | Brain - physiopathology | Tauopathies - classification | Supranuclear Palsy, Progressive - classification | Tauopathies - diagnosis | Brain - pathology | Inclusion Bodies - pathology | Aged | Motor Neuron Disease - diagnosis | Supranuclear Palsy, Progressive - diagnosis | Motor Neuron Disease - classification | Index Medicus | Abridged Index Medicus
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8. Full Text Loss of motoneuron-specific microRNA-218 causes systemic neuromuscular failure
by Amin, Neal D and Bai, Ge and Klug, Jason R and Bonanomi, Dario and Pankratz, Matthew T and Gifford, Wesley D and Hinckley, Christopher A and Sternfeld, Matthew J and Driscoll, Shawn P and Dominguez, Bertha and Lee, Kuo-Fen and Jin, Xin and Pfaff, Samuel L
Science, ISSN 0036-8075, 12/2015, Volume 350, Issue 6267, pp. 1525 - 1529
Dysfunction of microRNA (miRNA) metabolism is thought to underlie diseases affecting motoneurons. One miRNA, miR-218, is abundantly and selectively expressed... 
MOTOR-NEURONS | HYPEREXCITABILITY | BIOGENESIS | MULTIDISCIPLINARY SCIENCES | IN-VIVO | MIR-218 | SPINAL-CORD | DROSHA | AMYOTROPHIC-LATERAL-SCLEROSIS | FATE | BINDING | Motor Neurons - physiology | Neurodegenerative Diseases - pathology | Spinal Cord - metabolism | Gene Expression Regulation | Neurodegenerative Diseases - genetics | Gene Regulatory Networks | Motor Neurons - pathology | Mice, Knockout | Motor Neurons - metabolism | Animals | Motor Neuron Disease - genetics | Mice | MicroRNAs - genetics | Spinal Cord - physiopathology | MicroRNAs - physiology | Motor Neuron Disease - physiopathology | MicroRNA | Motor neurons | Neuromuscular diseases | Genetic aspects | Health aspects | Risk factors | Index Medicus | Networks | Ribonucleic acids | Neurons | Genes | Motors | Failure | Diseases
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9. Full Text Experimental transmissibility of mutant SOD1 motor neuron disease
by Ayers, Jacob I and Fromholt, Susan and Koch, Morgan and DeBosier, Adam and McMahon, Ben and Xu, Guilian and Borchelt, David R
Acta Neuropathologica, ISSN 0001-6322, 12/2014, Volume 128, Issue 6, pp. 791 - 803
By unknown mechanisms, the symptoms of amyotrophic lateral sclerosis (ALS) seem to spread along neuroanatomical pathways to engulf the motor nervous system.... 
Pathology | Neurosciences | Transmissibility | Medicine & Public Health | Superoxide dismutase 1 | Motor neuron disease | Amyotrophic lateral sclerosis | Seeding | ALS-LINKED SOD1 | AMYOTROPHIC-LATERAL-SCLEROSIS | PATHOLOGY | NEUROSCIENCES | FAMILIAL ALS | CLINICAL NEUROLOGY | SCRAPIE | WILD-TYPE SOD1 | PRION PROTEINS | SUPEROXIDE-DISMUTASE | MINK ENCEPHALOPATHY | AGGREGATION | TRANSGENIC MICE | Superoxide Dismutase - genetics | Amyotrophic Lateral Sclerosis - physiopathology | Paralysis - metabolism | Spinal Cord - pathology | Inclusion Bodies - metabolism | Motor Neuron Disease - physiopathology | Superoxide Dismutase - metabolism | Disease Models, Animal | Animals, Newborn | Genetic Predisposition to Disease | Mice, Inbred C57BL | Bacterial Proteins - genetics | Kaplan-Meier Estimate | Mice, Transgenic | Mice, Inbred C3H | Motor Neuron Disease - pathology | Animals | Inclusion Bodies - pathology | Bacterial Proteins - metabolism | Luminescent Proteins - genetics | Spinal Cord - physiopathology | Mutation | Superoxide Dismutase-1 | Microscopy, Fluorescence | Luminescent Proteins - metabolism | Proteins | Development and progression | Superoxide | Genetic engineering | Neurons | Index Medicus
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10. Full Text Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF
by Xu, Lan and Pun, San and Saxena, Smita and Santos, Alexandre Ferrão and Caroni, Pico
Nature Neuroscience, ISSN 1097-6256, 03/2006, Volume 9, Issue 3, pp. 408 - 419
Neurodegenerative diseases can have long preclinical phases and insidious progression patterns, but the mechanisms of disease progression are poorly... 
UBIQUITIN-PROTEASOME SYSTEM | ALZHEIMERS-DISEASE | MUSCLE-FIBERS | LINKED SOD1 MUTANTS | ALS MICE | AMYOTROPHIC-LATERAL-SCLEROSIS | MOTOR-NEURON DISEASE | MUTANT SUPEROXIDE-DISMUTASE | CILIARY NEUROTROPHIC FACTOR | TRANSGENIC MOUSE MODEL | NEUROSCIENCES | Superoxide Dismutase - genetics | Synaptic Vesicles - metabolism | Neuromuscular Junction - metabolism | Humans | Nerve Degeneration - physiopathology | Nerve Degeneration - genetics | Muscle, Skeletal - innervation | Motor Neurons - pathology | Neuronal Plasticity - genetics | Motor Neuron Disease - genetics | Muscle Fatigue - drug effects | Motor Neuron Disease - drug therapy | Motor Neuron Disease - physiopathology | Ciliary Neurotrophic Factor - pharmacology | Motor Neurons - drug effects | Disease Models, Animal | Genetic Predisposition to Disease - genetics | Synaptic Vesicles - ultrastructure | Axons - drug effects | Axons - metabolism | Ciliary Neurotrophic Factor - therapeutic use | Mice, Transgenic | Mutation - genetics | Neuromuscular Junction - ultrastructure | Motor Neurons - metabolism | Animals | Muscle Fibers, Fast-Twitch - physiology | Muscle Fibers, Slow-Twitch - physiology | Axons - pathology | Muscle, Skeletal - physiopathology | Muscle Fatigue - genetics | Mice | Superoxide Dismutase-1 | Nerve Degeneration - drug therapy | Axons | Brain research | Physiological aspects | Nervous system | Degeneration | Research | Ciliary neurotrophic factor | Identification and classification | Index Medicus
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11. Full Text Emerging Links between Lipid Droplets and Motor Neuron Diseases
by Pennetta, Giuseppa and Welte, Michael A
Developmental Cell, ISSN 1534-5807, 05/2018, Volume 45, Issue 4, pp. 427 - 432
Lipid droplets (LDs) are ubiquitous fat storage organelles and play key roles in lipid metabolism and energy homeostasis; in addition, they contribute to... 
CLEARANCE | TRANSPORT | MEMBRANE | ALS | DEVELOPMENTAL BIOLOGY | ACCUMULATION | PROTEINS | RECEPTORS | ASSOCIATION | PROTECTS | CELL BIOLOGY | Lipids - chemistry | Humans | Lipid Droplets - physiology | Homeostasis | Lipid Metabolism | Motor Neuron Disease - physiopathology | Organelles - metabolism | Lipolysis | Medicine, Experimental | Medical research | Neurons | Index Medicus
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12. Full Text Novel threshold tracking techniques suggest that cortical hyperexcitability is an early feature of motor neuron disease
by Vucic, Steve and Kiernan, Matthew C
Brain, ISSN 0006-8950, 09/2006, Volume 129, Issue 9, pp. 2436 - 2446
The dying forward hypothesis of motor neuron disease (MND) suggests that corticomotoneurons induce excitotoxic anterior horn cell death, with involvement of... 
Threshold tracking | MND | Cortical hyperexcitability | STRENGTH-DURATION PROPERTIES | AXONAL EXCITABILITY | HUMAN PERIPHERAL-NERVE | SILENT PERIOD | AMYOTROPHIC-LATERAL-SCLEROSIS | NEUROSCIENCES | cortical hyperexcitability | CLINICAL NEUROLOGY | EXCITABILITY PROPERTIES | CORTICOCORTICAL INHIBITION | threshold tracking | TRANSCRANIAL MAGNETIC STIMULATION | BRAIN-STIMULATION | INTRACORTICAL INHIBITION | Motor Neurons - physiology | Neural Inhibition | Evoked Potentials, Motor - physiology | Humans | Middle Aged | Male | Motor Cortex - physiopathology | Axons - physiology | Transcranial Magnetic Stimulation - methods | Action Potentials - physiology | Cell Death - physiology | Median Nerve - physiopathology | Muscle Contraction - physiology | Muscle, Skeletal - physiopathology | Adult | Female | Aged | Motor Neuron Disease - physiopathology | Electric Stimulation - methods | Index Medicus | Abridged Index Medicus
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13. Full Text Finger extension weakness and downbeat nystagmus motor neuron disease syndrome: A novel motor neuron disorder?
by Delva, Aline and Thakore, Nimish and Pioro, Erik P and Poesen, Koen and Saunders‐Pullman, Rachel and Meijer, Inge A and Rucker, Janet C and Kissel, John T and Van Damme, Philip
Muscle & Nerve, ISSN 0148-639X, 12/2017, Volume 56, Issue 6, pp. 1164 - 1168
Introduction : Disturbances of eye movements are infrequently encountered in motor neuron diseases (MNDs) or motor neuropathies, and there is no known syndrome... 
motor neuron disease | finger extension | downbeat nystagmus | FEWDON‐MND | muscle wasting | FEWDON-MND | NEUROSCIENCES | CLINICAL NEUROLOGY | Motor Neuron Disease - diagnostic imaging | Motor Neuron Disease - complications | Humans | Muscle Weakness - complications | Male | Fingers - physiopathology | Nystagmus, Pathologic - physiopathology | Electrodiagnosis - methods | Young Adult | Muscle Weakness - diagnostic imaging | Nystagmus, Pathologic - complications | Adolescent | Adult | Female | Retrospective Studies | Muscle Weakness - physiopathology | Motor Neuron Disease - physiopathology | Nystagmus, Pathologic - diagnostic imaging | Medical research | Nervous system diseases | Neurons | Nystagmus | Government finance | Medicine, Experimental | Development and progression | Eye | Etiology | Finger | Motor neuron disease | Muscles | Eye movements | Patients | Index Medicus | Short Reports | Short Report
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