Amyotrophic Lateral Sclerosis

Red cell superoxide dismutase activity in sporadic amyotrophic lateral sclerosis

Clinical Neuroscience / Amyotrophic Lateral Sclerosis / Humans / Mutation / Female / Clinical / Male / Differential Diagnosis / Spectrophotometry / Superoxide Dismutase / Clinical Sciences / Middle Aged / Adult / Prognosis / Biological markers / Enzyme activity / Disease Progression / Erythrocytes / Healthy Subjects / Neurosciences / Predictive value of tests / Control Group / Diagnostic Criteria / Clinical / Male / Differential Diagnosis / Spectrophotometry / Superoxide Dismutase / Clinical Sciences / Middle Aged / Adult / Prognosis / Biological markers / Enzyme activity / Disease Progression / Erythrocytes / Healthy Subjects / Neurosciences / Predictive value of tests / Control Group / Diagnostic Criteria

Loss of Prion Protein in a Transgenic Model of Amyotrophic Lateral Sclerosis

Cognitive Science / Protein Folding / Skeletal muscle biology / Motor neuron / Transgenic Mice / Amyotrophic Lateral Sclerosis / Molecular chaperones / Molecular and cellular biology / Genetic Testing / Mutation / Mice / Animals / Spinal Cord / Superoxide Dismutase / Western blot / Sciatic Nerve / Cellular and Molecular Neuroscience / Enzymatic Activity / Neurosciences / Down-Regulation / Prion Protein / DNA mutational analysis / Amyotrophic Lateral Sclerosis / Molecular chaperones / Molecular and cellular biology / Genetic Testing / Mutation / Mice / Animals / Spinal Cord / Superoxide Dismutase / Western blot / Sciatic Nerve / Cellular and Molecular Neuroscience / Enzymatic Activity / Neurosciences / Down-Regulation / Prion Protein / DNA mutational analysis

C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking

Mass Spectrometry / Amyotrophic Lateral Sclerosis / Biological Sciences / Humans / Mice / Animals / Human Molecular Genetics / Proteins / Frontotemporal Dementia / Endosomes / Animals / Human Molecular Genetics / Proteins / Frontotemporal Dementia / Endosomes

Destabilization of apoprotein is insufficient to explain Cu,Zn-superoxide dismutase-linked ALS pathogenesis

Mass Spectrometry / Multidisciplinary / Amyotrophic Lateral Sclerosis / Humans / Mutation / Differential scanning calorimetry / Superoxide Dismutase / Metals / Glass Transition Temperature / Amino Acid Substitution Rates / Transition Temperature / Differential scanning calorimetry / Superoxide Dismutase / Metals / Glass Transition Temperature / Amino Acid Substitution Rates / Transition Temperature

Vascular Endothelial Growth Factor Prevents Paralysis and Motoneuron Death in a Rat Model of Excitotoxic Spinal Cord Neurodegeneration

Neurodegeneration / Neurodegenerative Diseases / Amyotrophic Lateral Sclerosis / VEGF / Spinal Cord / Neurodegenerative disorders / Motoneurons / Neurodegenerative disorders / Motoneurons

Optimizing separation efficiency of 2-DE procedures for visualization of different superoxide dismutase forms in a cellular model of amyotrophic lateral sclerosis

Chemical Engineering / Analytical Chemistry / Amyotrophic Lateral Sclerosis / Humans / Mutation / Superoxide Dismutase / Neuroblastoma / Cellular Automaton Model / Electrophoresis / Isoenzymes / Reproducibility of Results / Transfection / Two-Dimensional Gel Electrophoresis / Sensitivity and Specificity / Oxidation-Reduction / Biochemistry and cell biology / Superoxide Dismutase / Neuroblastoma / Cellular Automaton Model / Electrophoresis / Isoenzymes / Reproducibility of Results / Transfection / Two-Dimensional Gel Electrophoresis / Sensitivity and Specificity / Oxidation-Reduction / Biochemistry and cell biology

Amyotrophic lateral sclerosis: a long preclinical period?

Amyotrophic Lateral Sclerosis / Humans / Mice / Animals / Synaptic Transmission / Risk factors / Time Factors / Risk Factors / Prodromal Symptoms / Risk factors / Time Factors / Risk Factors / Prodromal Symptoms

Genetically modified human umbilical cord blood cells expressing vascular endothelial growth factor and fibroblast growth factor 2 differentiate into glial cells after transplantation into amyotrophic lateral sclerosis transgenic mice

Amyotrophic Lateral Sclerosis / Hematopoietic Stem Cells / Humans / Mice / Electroporation / Animals / Fibroblast Growth Factor / Fluorescent Antibody Technique / Experimental / Animals / Fibroblast Growth Factor / Fluorescent Antibody Technique / Experimental

Partial deficiency of manganese superoxide dismutase exacerbates a transgenic mouse model of amyotrophic lateral sclerosis

Oxidative Stress / Amyotrophic Lateral Sclerosis / Humans / Mice / Female / Animals / Male / Cell Death / Substantia nigra / Superoxide Dismutase / Phenotype / Clinical Sciences / Transgenic Mouse Technology / Neurosciences / Animals / Male / Cell Death / Substantia nigra / Superoxide Dismutase / Phenotype / Clinical Sciences / Transgenic Mouse Technology / Neurosciences

Distal axonopathy in an alsin-deficient mouse model

Motor neuron / Amyotrophic Lateral Sclerosis / Biological Sciences / Brain / Pregnancy / Humans / Mutation / Mice / Female / Animals / Spinal Cord / Male / Human Molecular Genetics / Central Nervous System / Corticospinal Tract / Superoxide Dismutase / Mouse Model / Amino Acid Sequence / Axons / Humans / Mutation / Mice / Female / Animals / Spinal Cord / Male / Human Molecular Genetics / Central Nervous System / Corticospinal Tract / Superoxide Dismutase / Mouse Model / Amino Acid Sequence / Axons

Progranulin genetic variability contributes to amyotrophic lateral sclerosis

Cognitive Science / Neurology / Dementia / Belgium / Amyotrophic Lateral Sclerosis / Humans / Genetic Testing / Mutation / Haplotypes / Female / Male / Netherlands / Clinical Sciences / Aged / Middle Aged / Genotype / Adult / Survival Rate / Genetic variation / Genetic Variability / Genetic Markers / Neurosciences / Age of Onset / Humans / Genetic Testing / Mutation / Haplotypes / Female / Male / Netherlands / Clinical Sciences / Aged / Middle Aged / Genotype / Adult / Survival Rate / Genetic variation / Genetic Variability / Genetic Markers / Neurosciences / Age of Onset

Frontotemporal dementia–amyotrophic lateral sclerosis syndrome locus on chromosome 16p12.1–q12.2: genetic, clinical and neuropathological analysis

Cognitive Science / Magnetic Resonance Imaging / Positron Emission Tomography / Amyotrophic Lateral Sclerosis / Brain / Humans / Haplotypes / Female / Male / Iron / Differential Diagnosis / Electromyography / Phosphorylation / Pedigree / Genetic linkage analysis / Clinical Sciences / Aged / Middle Aged / Frontotemporal Dementia / DNA binding proteins / Neurosciences / Humans / Haplotypes / Female / Male / Iron / Differential Diagnosis / Electromyography / Phosphorylation / Pedigree / Genetic linkage analysis / Clinical Sciences / Aged / Middle Aged / Frontotemporal Dementia / DNA binding proteins / Neurosciences

Novel dimeric interface and electrostatic recognition in bacterial Cu,Zn superoxide dismutase

Protein Folding / Multidisciplinary / Macromolecular X-Ray Crystallography / Amyotrophic Lateral Sclerosis / Free Radical / Humans / Differential scanning calorimetry / Escherichia coli / Animals / Fishes / Superoxide Dismutase / Enzyme / Cattle / Antioxidant enzyme / Molecular cloning / Active site / Protein Secondary Structure Prediction / Neurodegenerative Disease / Amino Acid Sequence / Recombinant Proteins / Dimerization / Disulfide bond / Photobacterium / Humans / Differential scanning calorimetry / Escherichia coli / Animals / Fishes / Superoxide Dismutase / Enzyme / Cattle / Antioxidant enzyme / Molecular cloning / Active site / Protein Secondary Structure Prediction / Neurodegenerative Disease / Amino Acid Sequence / Recombinant Proteins / Dimerization / Disulfide bond / Photobacterium

Aggregation propensities of superoxide dismutase G93 hotspot mutants mirror ALS clinical phenotypes

Small angle X-ray and neutron scattering / Multidisciplinary / Macromolecular X-Ray Crystallography / Amyotrophic Lateral Sclerosis / Humans / Mutation / Copper / Superoxide Dismutase / Phenotype / Solutions / Acids / Edetic Acid / Mutation / Copper / Superoxide Dismutase / Phenotype / Solutions / Acids / Edetic Acid

Progranulin genetic variability contributes to amyotrophic lateral sclerosis

Cognitive Science / Neurology / Dementia / Belgium / Amyotrophic Lateral Sclerosis / Humans / Genetic Testing / Mutation / Haplotypes / Female / Male / Netherlands / Clinical Sciences / Aged / Middle Aged / Genotype / Adult / Survival Rate / Genetic variation / Genetic Variability / Genetic Markers / Neurosciences / Age of Onset / Humans / Genetic Testing / Mutation / Haplotypes / Female / Male / Netherlands / Clinical Sciences / Aged / Middle Aged / Genotype / Adult / Survival Rate / Genetic variation / Genetic Variability / Genetic Markers / Neurosciences / Age of Onset
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