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Nature Neuroscience, ISSN 1097-6256, 11/2012, Volume 15, Issue 11, pp. 1488 - 1497
FUS/TLS (fused in sarcoma/translocated in liposarcoma) and TDP-43 are integrally involved in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia.... 
NEURODEGENERATIVE DISEASE | GENE | AMYOTROPHIC-LATERAL-SCLEROSIS | FAMILY PROTEINS | FUS PATHOLOGY | MUTATIONS | FRONTOTEMPORAL LOBAR DEGENERATION | BINDING | NEUROSCIENCES | BRAIN | NASCENT TRANSCRIPTION | RNA, Small Interfering - genetics | Protein Binding - genetics | Oligonucleotide Array Sequence Analysis | Humans | tau Proteins - metabolism | Gene Expression Profiling | RNA, Messenger - metabolism | Kv Channel-Interacting Proteins - metabolism | Brain - metabolism | Frontotemporal Dementia - metabolism | RNA Splicing - genetics | Frontotemporal Dementia - genetics | RNA-Binding Protein FUS - deficiency | Amyotrophic Lateral Sclerosis - genetics | Cell Cycle Proteins - metabolism | Ubiquitin-Protein Ligases - metabolism | RNA-Binding Protein FUS - genetics | Mice, Knockout | Motor Neurons - metabolism | Amyotrophic Lateral Sclerosis - pathology | Shal Potassium Channels - metabolism | Brain - pathology | Mice | Neurofilament Proteins - metabolism | RNA, Small Interfering - metabolism | Immunoprecipitation | Spinal Cord - metabolism | DNA-Binding Proteins - deficiency | DNA-Binding Proteins - metabolism | tau Proteins - genetics | Cell Cycle Proteins - genetics | Female | RNA Precursors - metabolism | Excitatory Amino Acid Transporter 2 - genetics | Membrane Proteins - metabolism | Frontotemporal Dementia - pathology | Gene Expression Regulation - genetics | Mice, Inbred C57BL | RNA, Messenger - genetics | RNA Precursors - genetics | Protein Structure, Tertiary - genetics | RNA-Binding Protein FUS - metabolism | DNA-Binding Proteins - genetics | Excitatory Amino Acid Transporter 2 - metabolism | Nerve Tissue Proteins - genetics | Nerve Tissue Proteins - metabolism | Carrier Proteins - genetics | Animals | Carrier Proteins - metabolism | Histone-Lysine N-Methyltransferase - metabolism | Amyotrophic Lateral Sclerosis - metabolism | Neural Cell Adhesion Molecules - metabolism | Neural Stem Cells - metabolism | Cell Line, Transformed | Amyotrophic lateral sclerosis | Development and progression | Genetic aspects | Messenger RNA | Health aspects | Index Medicus
Journal Article
Acta Neuropathologica, ISSN 0001-6322, 11/2012, Volume 124, Issue 5, pp. 733 - 747
While the pathogenesis of amyotrophic lateral sclerosis (ALS) remains to be clearly delineated, there is mounting evidence that altered RNA metabolism is a... 
Pathology | Neurosciences | Ubiquitination | Medicine & Public Health | Amyotrophic lateral sclerosis | Superoxide dismutase | RGNEF | C9orf72 | RNA binding proteins | HEXANUCLEOTIDE REPEAT EXPANSION | GENE-MUTATIONS | INCLUSIONS | AMYOTROPHIC-LATERAL-SCLEROSIS | PATHOLOGY | FRONTOTEMPORAL LOBAR DEGENERATION | LENGTH POLYGLUTAMINE EXPANSIONS | NEUROSCIENCES | CLINICAL NEUROLOGY | MESSENGER-RNA | PATHOLOGICAL TDP-43 | EXPRESSION | SOD1 MUTATIONS | Superoxide Dismutase - genetics | Immunoprecipitation | Membrane Glycoproteins - metabolism | Organic Chemicals | Sequestosome-1 Protein | Humans | Male | Transcription Factor TFIIIA - metabolism | DNA-Binding Proteins - metabolism | Guanine Nucleotide Exchange Factors - metabolism | Spinal Cord - pathology | Female | C9orf72 Protein | Superoxide Dismutase - metabolism | Peripherins | Gene Expression Regulation - genetics | Amyotrophic Lateral Sclerosis - genetics | RNA-Binding Protein FUS - genetics | RNA-Binding Protein FUS - metabolism | DNA-Binding Proteins - genetics | Mutation - genetics | Motor Neurons - metabolism | Nerve Tissue Proteins - metabolism | Proteins - genetics | Microscopy, Confocal | Amyotrophic Lateral Sclerosis - pathology | Proteins - metabolism | Amyotrophic Lateral Sclerosis - classification | Superoxide Dismutase-1 | Adaptor Proteins, Signal Transducing - metabolism | Neurofilament Proteins - metabolism | Intermediate Filament Proteins - metabolism | RNA-Binding Proteins - metabolism | Immunohistochemistry | Ubiquitin | RNA | Neurons | Chemical properties | Binding proteins | Intermediate filament proteins | Protein binding | Index Medicus | Motor neurons | Antibodies | Colorimetry | Nucleotides | Metabolism | FUS protein | RNA-binding protein | Inclusion bodies | Neurofilaments | Peripherin | Mutation
Journal Article
Journal of Biological Chemistry, ISSN 0021-9258, 04/2015, Volume 290, Issue 15, pp. 9753 - 9766
Journal Article
Brain Research, ISSN 0006-8993, 2009, Volume 1305, pp. 168 - 182
Journal Article
Journal of Neuroscience Research, ISSN 0360-4012, 08/2017, Volume 95, Issue 8, pp. 1647 - 1665
Glycoprotein nonmetastatic melanoma protein B (GPNMB) aggregates are observed in the spinal cord of amyotrophic lateral sclerosis (ALS) patients, but the... 
Transactive response DNA binding protein 43kDa (TDP‐43) | Glycoprotein nonmetastatic melanoma protein B (GPNMB) | Amyotrophic lateral sclerosis (ALS) | Sporadic ALS patient | mouse motor neuron cells (NSC34) | Transactive response DNA binding protein 43kDa (TDP-43) | HUMAN TDP-43 | ALS | MELANOMA PROTEIN-B | AMYOTROPHIC-LATERAL-SCLEROSIS | NEUROSCIENCES | SKELETAL-MUSCLE | IN-VITRO | NEUROPROTECTIVE FACTOR | PRESYMPTOMATIC TRANSGENIC MICE | SOD1 GENE | EARLY DECREASE | Membrane Glycoproteins - metabolism | Microtubule-Associated Proteins - metabolism | Humans | Male | Glial Fibrillary Acidic Protein - metabolism | DNA-Binding Proteins - metabolism | MAP Kinase Signaling System - genetics | Cell Death - genetics | Protein Aggregates - genetics | Spinal Cord - pathology | Female | Proto-Oncogene Proteins c-akt - metabolism | Motor Neurons - physiology | Gene Expression Regulation - genetics | Cells, Cultured | Signal Transduction - genetics | DNA-Binding Proteins - genetics | Mutation - genetics | Motor Neurons - metabolism | Amyotrophic Lateral Sclerosis - pathology | Animals | MAP Kinase Signaling System - drug effects | Signal Transduction - physiology | Aged | Mice | Neurofilament Proteins - metabolism | Medical research | Nervous system diseases | Neurons | Cell death | Melanoma | Medicine, Experimental | Amyotrophic lateral sclerosis | Biochemistry | Protein binding | Spinal cord | Calcium | AKT protein | Motor task performance | Neuronal-glial interactions | Sclerosis | Proteins | Transfection | Rodents | Microtubule-associated protein 2 | Localization | Deoxyribonucleic acid--DNA | Recombinant | Binding | Stresses | Neurodegenerative diseases | Glial fibrillary acidic protein | Mortality | Glycoprotein | Extracellular signal-regulated kinase | Protein B | Neurofilament H | Patients | Stress | Microglia | Neurological diseases | Aggregates | Plasmids | Mutation | Position (location) | Apoptosis | Index Medicus
Journal Article
Journal of Neuroscience, ISSN 0270-6474, 09/2006, Volume 26, Issue 39, pp. 10006 - 10019
alpha-Internexin, a neuronal intermediate filament protein implicated in neurodegenerative disease, coexists with the neurofilament (NF) triplet proteins... 
Neurofilament | α-internexin | Tropical spastic paraparesis | Cytoskeleton | Neurofilament inclusion disease | Axonal transport | Intermediate filament | neurofilament inclusion disease | SLOW AXONAL-TRANSPORT | MARIE-TOOTH-DISEASE | cytoskeleton | CELL-SURFACE EXPRESSION | tropical spastic paraparesis | AMYOTROPHIC-LATERAL-SCLEROSIS | MOTOR-NEURON DISEASE | NEUROSCIENCES | INTERMEDIATE-FILAMENT PROTEIN | intermediate filament | NF-L | INCLUSION DISEASE | neurofilament | axonal transport | alpha-internexin | TRANSGENIC MICE | Intermediate Filament Proteins - ultrastructure | Multiprotein Complexes | Recombinant Fusion Proteins - analysis | Recombinant Fusion Proteins - physiology | Male | Structure-Activity Relationship | Spinal Cord - ultrastructure | Microscopy, Immunoelectron | Nerve Degeneration - metabolism | Neurofilament Proteins - genetics | Spinal Cord - chemistry | Transfection | Neurofilament Proteins - analysis | Axons - ultrastructure | Intermediate Filament Proteins - genetics | Female | Retinal Ganglion Cells - chemistry | Retinal Ganglion Cells - ultrastructure | Mice, Inbred C57BL | Intermediate Filament Proteins - physiology | Rats | Axons - chemistry | Intermediate Filament Proteins - analysis | Nerve Degeneration - pathology | Intermediate Filaments - chemistry | Mice, Knockout | Protein Interaction Mapping | Protein Transport | Microscopy, Confocal | Animals | Neurofilament Proteins - physiology | Intermediate Filament Proteins - deficiency | Intermediate Filaments - ultrastructure | Neurofilament Proteins - deficiency | Neurofilament Proteins - ultrastructure | Mice | Microscopy, Fluorescence | Crosses, Genetic | Index Medicus
Journal Article
Nature Neuroscience, ISSN 1097-6256, 11/2003, Volume 6, Issue 11, pp. 1162 - 1168
The generation of neurons from stem cells involves the activity of proneural basic helix-loop-helix (bHLH) proteins, but the mechanism by which these proteins... 
VENTRAL NEURAL-TUBE | SOX2 | NERVOUS-SYSTEM | STEM-CELLS | BHLH PROTEIN | GENE-EXPRESSION | NEURONAL DIFFERENTIATION | CHICK-EMBRYO | ACHAETE-SCUTE | FATE | NEUROSCIENCES | Helix-Loop-Helix Motifs - physiology | Homeodomain Proteins - metabolism | PAX7 Transcription Factor | Phosphopyruvate Hydratase - metabolism | DNA-Binding Proteins - metabolism | Stem Cells | Tubulin - metabolism | Time Factors | Gene Expression Regulation, Developmental | Neurons - metabolism | Bromodeoxyuridine - metabolism | Membrane Glycoproteins | Spinal Cord - cytology | SOXB1 Transcription Factors | Cell Differentiation - physiology | Basic Helix-Loop-Helix Transcription Factors | DNA-Binding Proteins - physiology | HMGB Proteins | High Mobility Group Proteins - metabolism | Drosophila | Embryonic Induction | Neuropeptides - metabolism | Nuclear Proteins - metabolism | Electroporation - methods | Chick Embryo | Proto-Oncogene Proteins c-myc - metabolism | Nerve Tissue Proteins - metabolism | Transcription Factors - metabolism | Spinal Cord - embryology | Vertebrates | Animals | Eye Proteins - metabolism | Spinal Cord - physiology | Avian Proteins | LIM-Homeodomain Proteins | High Mobility Group Proteins - physiology | Mice | Nuclear Proteins - physiology | Neurofilament Proteins - metabolism | Physiological aspects | Research | DNA binding proteins | Neurons | Cell cycle | Index Medicus
Journal Article
Journal Article
Journal Article
Journal of Neuroscience, ISSN 0270-6474, 05/2009, Volume 29, Issue 18, pp. 5758 - 5767
Many neurodegenerative diseases exhibit axonal pathology, transport defects, and aberrant phosphorylation and aggregation of the microtubule binding protein... 
NEURODEGENERATIVE DISORDERS | HEAVY-CHAIN | DEMENTIA | PHOSPHORYLATION | ALZHEIMERS-DISEASE | SPINAL-CORD | COGNITIVE IMPAIRMENT | AMYLOID PRECURSOR PROTEIN | C-JUN | NEUROSCIENCES | TRANSGENIC MICE | Age Factors | Microtubule-Associated Proteins - metabolism | tau Proteins - metabolism | JNK Mitogen-Activated Protein Kinases - metabolism | Neurons - cytology | Green Fluorescent Proteins - genetics | Mitochondrial Proteins - genetics | Microscopy, Electron, Scanning - methods | Neurons - ultrastructure | Organelles - ultrastructure | Transfection - methods | tau Proteins - genetics | Mitochondrial Proteins - metabolism | Amyloid beta-Protein Precursor - metabolism | Axons - ultrastructure | Statistics, Nonparametric | Microtubule-Associated Proteins - deficiency | Animals, Newborn | Stress, Physiological - physiology | Mice, Inbred C57BL | Cells, Cultured | Axons - metabolism | Cytoskeleton - ultrastructure | Hippocampus - cytology | Protein Transport - genetics | Mice, Knockout | Nerve Tissue Proteins - metabolism | Kymography - methods | Amyloid beta-Protein Precursor - genetics | Animals | Adaptor Proteins, Signal Transducing - genetics | Cytoskeleton - metabolism | Luminescent Proteins - genetics | Mice | Adaptor Proteins, Signal Transducing - metabolism | Neurofilament Proteins - metabolism | Organelles - metabolism | Index Medicus
Journal Article
Journal of Neuroscience, ISSN 0270-6474, 06/2012, Volume 32, Issue 26, pp. 8855 - 8864
Wrapping of the myelin sheath around axons by oligodendrocytes is critical for the rapid conduction of electrical signals required for the normal functioning... 
PROTEOLIPID PROTEIN | REGULATED KINASE | PROGENITOR CELLS | NEUROTROPHIC FACTOR | AXONS MODULATE | SHEATH THICKNESS | LINEAGE CELLS | CENTRAL-NERVOUS-SYSTEM | BASAL FOREBRAIN | BASIC-PROTEIN | NEUROSCIENCES | Oligodendroglia - metabolism | MAP Kinase Signaling System - physiology | Phosphorylation | Cell Proliferation | Spinal Cord - metabolism | Age Factors | Mitogen-Activated Protein Kinase 1 - deficiency | Male | RNA, Messenger - metabolism | Oligodendroglia - ultrastructure | Neurofilament Proteins - genetics | Neurons - ultrastructure | Myelin Proteolipid Protein - metabolism | Female | Cell Differentiation | Neurons - metabolism | Spinal Cord - cytology | Organ Culture Techniques | Microscopy, Electron, Transmission | Myelin Sheath - physiology | Myelin Basic Protein - metabolism | Cells, Cultured | Mice, Transgenic | Phosphoric Diester Hydrolases - genetics | Mutation - genetics | 2',3'-Cyclic Nucleotide 3'-Phosphodiesterase | Animals | Mitogen-Activated Protein Kinase 3 - metabolism | Myelin Proteolipid Protein - genetics | Myelin Sheath - genetics | Mice | Mitogen-Activated Protein Kinase 3 - deficiency | Myelin Sheath - ultrastructure | Neurofilament Proteins - metabolism | Proteoglycans - genetics | Antigens - genetics | Mitogen-Activated Protein Kinase 1 - metabolism | Index Medicus | myelin | oligodendrocyte
Journal Article
Neurobiology of Aging, ISSN 0197-4580, 2013, Volume 34, Issue 1, pp. 248 - 262
Abstract Amyotrophic lateral sclerosis (ALS) is an adult-onset progressive disorder of unknown etiology characterized by the selective degeneration of motor... 
Neurology | Internal Medicine | RNA stability | p190RhoGEF | NFL | Rho guanine nucleotide exchange factor | RNA binding protein | Amyotrophic lateral sclerosis | RGNEF | Protein aggregates | P190RhoGEF | MOTOR-NEURONS | 3'-UNTRANSLATED REGION | BINDING PROTEIN P62 | ALS-LINKED SOD1 | FRONTOTEMPORAL LOBAR DEGENERATION | NEUROFILAMENT LIGHT SUBUNIT | NEUROSCIENCES | GERIATRICS & GERONTOLOGY | TDP-43 | HEXANUCLEOTIDE REPEAT | TRANSGENIC MICE | RNA, Small Interfering - genetics | Protein Binding - genetics | Immunoprecipitation | Sequestosome-1 Protein | Humans | Middle Aged | Ubiquitin - metabolism | Male | Green Fluorescent Proteins - genetics | RNA, Messenger - metabolism | DNA-Binding Proteins - metabolism | Neurofilament Proteins - genetics | Transfection | Guanine Nucleotide Exchange Factors - metabolism | Aged, 80 and over | Female | Inclusion Bodies - metabolism | Green Fluorescent Proteins - metabolism | Guanine Nucleotide Exchange Factors - genetics | Rho Guanine Nucleotide Exchange Factors | RNA Stability - genetics | Amyotrophic Lateral Sclerosis - pathology | Animals | Inclusion Bodies - pathology | Aged | Mice | Adaptor Proteins, Signal Transducing - metabolism | Cell Line, Transformed | RNA, Small Interfering - metabolism | Ubiquitin | Messenger RNA | Neurons | Analysis | Football (Professional) | Development and progression | Binding proteins | Guanine | Protein binding | Index Medicus | Animal models | Motor neuron disease | Nervous system | mRNA | Metabolism | Protein turnover | Signal transduction | RNA-binding protein | DNA-binding protein | guanine nucleotide exchange factor | Etiology | Inclusion bodies | Aging | Neurofilament protein | Neurofilaments | Degeneration
Journal Article