Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions.

PubWeight™: 2.08‹?› | Rank: Top 2%

🔗 View Article (PMC 39778)

Published in Proc Natl Acad Sci U S A on April 02, 1996

Authors

P H Tu1, P Raju, K A Robinson, M E Gurney, J Q Trojanowski, V M Lee

Author Affiliations

1: Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, 19104-4283, USA.

Articles citing this

Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS). Proc Natl Acad Sci U S A (2002) 4.37

Defects in regulation of apoptosis in caspase-2-deficient mice. Genes Dev (1998) 3.24

Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2000) 3.11

Neurofilaments and orthograde transport are reduced in ventral root axons of transgenic mice that express human SOD1 with a G93A mutation. J Cell Biol (1997) 1.95

Intrathecal infusion of a Ca(2+)-permeable AMPA channel blocker slows loss of both motor neurons and of the astrocyte glutamate transporter, GLT-1 in a mutant SOD1 rat model of ALS. Exp Neurol (2007) 1.56

Molecular and cellular pathways of neurodegeneration in motor neurone disease. J Neurol Neurosurg Psychiatry (2005) 1.55

The Environmental Genome Project: functional analysis of polymorphisms. Environ Health Perspect (1998) 1.46

Modeling ALS with iPSCs reveals that mutant SOD1 misregulates neurofilament balance in motor neurons. Cell Stem Cell (2014) 1.39

The roles of ASK family proteins in stress responses and diseases. Cell Commun Signal (2009) 1.32

Corticospinal motor neurons and related subcerebral projection neurons undergo early and specific neurodegeneration in hSOD1G⁹³A transgenic ALS mice. J Neurosci (2011) 1.29

Age-dependent induction of congophilic neurofibrillary tau inclusions in tau transgenic mice. Am J Pathol (2001) 1.28

SOD1 and amyotrophic lateral sclerosis: mutations and oligomerization. PLoS One (2008) 1.27

Mutant SOD1G93A triggers mitochondrial fragmentation in spinal cord motor neurons: neuroprotection by SIRT3 and PGC-1α. Neurobiol Dis (2012) 1.26

Absence of neurofilaments reduces the selective vulnerability of motor neurons and slows disease caused by a familial amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutant. Proc Natl Acad Sci U S A (1998) 1.23

Catalase and glutathione peroxidase mimics. Biochem Pharmacol (2008) 1.19

Altered axonal architecture by removal of the heavily phosphorylated neurofilament tail domains strongly slows superoxide dismutase 1 mutant-mediated ALS. Proc Natl Acad Sci U S A (2005) 1.19

Programmed cell death in amyotrophic lateral sclerosis. J Clin Invest (2003) 1.17

Late onset of motor neurons in mice overexpressing wild-type peripherin. J Cell Biol (1999) 1.16

Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase. Proc Natl Acad Sci U S A (1998) 1.11

Molecular factors underlying selective vulnerability of motor neurons to neurodegeneration in amyotrophic lateral sclerosis. J Neurol (2000) 1.10

Modulation of mutant superoxide dismutase 1 aggregation by co-expression of wild-type enzyme. J Neurochem (2008) 1.09

Calcium dynamics and buffering in oculomotor neurones from mouse that are particularly resistant during amyotrophic lateral sclerosis (ALS)-related motoneurone disease. J Physiol (2000) 1.08

Optimised and rapid pre-clinical screening in the SOD1(G93A) transgenic mouse model of amyotrophic lateral sclerosis (ALS). PLoS One (2011) 1.00

Early onset of severe familial amyotrophic lateral sclerosis with a SOD-1 mutation: potential impact of CNTF as a candidate modifier gene. Am J Hum Genet (2002) 1.00

Sensory involvement in the SOD1-G93A mouse model of amyotrophic lateral sclerosis. Exp Mol Med (2009) 0.96

A neurotoxic peripherin splice variant in a mouse model of ALS. J Cell Biol (2003) 0.95

Apoptotic death of neurons exhibiting peripherin aggregates is mediated by the proinflammatory cytokine tumor necrosis factor-alpha. J Cell Biol (2001) 0.92

Oxidative stress and adult neurogenesis--effects of radiation and superoxide dismutase deficiency. Semin Cell Dev Biol (2012) 0.92

Characterization of a novel SOD-1(G93A) transgenic mouse line with very decelerated disease development. PLoS One (2010) 0.92

A new zebrafish model produced by TILLING of SOD1-related amyotrophic lateral sclerosis replicates key features of the disease and represents a tool for in vivo therapeutic screening. Dis Model Mech (2013) 0.92

Slow development of ALS-like spinal cord pathology in mutant valosin-containing protein gene knock-in mice. Cell Death Dis (2012) 0.92

Discovery of transgene insertion sites by high throughput sequencing of mate pair libraries. BMC Genomics (2014) 0.90

Dimerization, oligomerization, and aggregation of human amyotrophic lateral sclerosis copper/zinc superoxide dismutase 1 protein mutant forms in live cells. J Biol Chem (2014) 0.86

Reduction of axonal caliber does not alleviate motor neuron disease caused by mutant superoxide dismutase 1. Proc Natl Acad Sci U S A (2000) 0.86

Ribonuclease 4 protects neuron degeneration by promoting angiogenesis, neurogenesis, and neuronal survival under stress. Angiogenesis (2012) 0.85

Amyotrophic lateral sclerosis is associated with hypolipidemia at the presymptomatic stage in mice. PLoS One (2011) 0.85

The type III neurofilament peripherin is expressed in the tuberomammillary neurons of the mouse. BMC Neurosci (2008) 0.85

Induced pluripotent stem cells from ALS patients for disease modeling. Brain Res (2014) 0.84

Strategic approaches to developing drug treatments for ALS. Drug Discov Today (2007) 0.84

Synaptic sprouting increases the uptake capacities of motoneurons in amyotrophic lateral sclerosis mice. Proc Natl Acad Sci U S A (2001) 0.84

Death receptor 6 (DR6) antagonist antibody is neuroprotective in the mouse SOD1G93A model of amyotrophic lateral sclerosis. Cell Death Dis (2013) 0.84

Transgenic mice for interleukin 3 develop motor neuron degeneration associated with autoimmune reaction against spinal cord motor neurons. Proc Natl Acad Sci U S A (1998) 0.83

A natural human IgM that binds to gangliosides is therapeutic in murine models of amyotrophic lateral sclerosis. Dis Model Mech (2015) 0.82

Amyotrophic lateral sclerosis model derived from human embryonic stem cells overexpressing mutant superoxide dismutase 1. Stem Cells Transl Med (2012) 0.80

A perspective on stem cell modeling of amyotrophic lateral sclerosis. Cell Cycle (2015) 0.79

Advanced glycation endproducts in neurofilament conglomeration of motoneurons in familial and sporadic amyotrophic lateral sclerosis. Mol Med (1998) 0.79

Intermittent hypoxia can aggravate motor neuronal loss and cognitive dysfunction in ALS mice. PLoS One (2013) 0.79

A truncating SOD1 mutation, p.Gly141X, is associated with clinical and pathologic heterogeneity, including frontotemporal lobar degeneration. Acta Neuropathol (2015) 0.78

A neuronal death model: overexpression of neuronal intermediate filament protein peripherin in PC12 cells. J Biomed Sci (2012) 0.78

Mutant CuZn superoxide dismutase in motor neuron disease. J Inherit Metab Dis (1998) 0.78

Mice with mutation in dynein heavy chain 1 do not share the same tau expression pattern with mice with SOD1-related motor neuron disease. Neurochem Res (2011) 0.77

Suppression of extensive neurofilament phosphorylation rescues α-Internexin/peripherin-overexpressing PC12 cells from neuronal cell death. PLoS One (2012) 0.77

Increased axonal ribosome numbers is an early event in the pathogenesis of amyotrophic lateral sclerosis. PLoS One (2014) 0.76

Dynactin Deficiency in the CNS of Humans with Sporadic ALS and Mice with Genetically Determined Motor Neuron Degeneration. Neurochem Res (2013) 0.76

A system for studying mechanisms of neuromuscular junction development and maintenance. Development (2016) 0.75

Stem cell factor-activated bone marrow ameliorates amyotrophic lateral sclerosis by promoting protective microglial migration. J Neurosci Res (2014) 0.75

Ablation of the cardiac ryanodine receptor phospho-site Ser2808 does not alter the adrenergic response or the progression to heart failure in mice. Elimination of the genetic background as critical variable. J Mol Cell Cardiol (2017) 0.75

Mutant Cu,Zn superoxide dismutase in motor neuron disease. Age (Omaha) (1998) 0.75

Bid Promotes K63-Linked Polyubiquitination of Tumor Necrosis Factor Receptor Associated Factor 6 (TRAF6) and Sensitizes to Mutant SOD1-Induced Proinflammatory Signaling in Microglia. eNeuro (2016) 0.75

Mechanisms of Muscle Denervation in Aging: Insights from a Mouse Model of Amyotrophic Lateral Sclerosis. Aging Dis (2015) 0.75

Neurofilament depletion improves microtubule dynamics via modulation of Stat3/stathmin signaling. Acta Neuropathol (2016) 0.75

Articles cited by this

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature (1993) 21.65

Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation. Science (1994) 18.08

An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron (1995) 6.03

Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase. Science (1993) 5.78

Cross-linker system between neurofilaments, microtubules, and membranous organelles in frog axons revealed by the quick-freeze, deep-etching method. J Cell Biol (1982) 5.51

Defective axonal transport in a transgenic mouse model of amyotrophic lateral sclerosis. Nature (1995) 2.83

Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (1995) 2.79

Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: a mouse model of amyotrophic lateral sclerosis. Cell (1993) 2.42

Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease. Cell (1993) 2.40

Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis. Am J Pathol (1994) 2.12

Neuropathological changes in two lines of mice carrying a transgene for mutant human Cu,Zn SOD, and in mice overexpressing wild type human SOD: a model of familial amyotrophic lateral sclerosis (FALS). Brain Res (1995) 1.97

Attenuation of focal cerebral ischemic injury in transgenic mice overexpressing CuZn superoxide dismutase. Proc Natl Acad Sci U S A (1991) 1.82

Amyotrophic lateral sclerosis: recent insights from genetics and transgenic mice. Cell (1995) 1.74

Phosphate dependent and independent neurofilament epitopes in the axonal swellings of patients with motor neuron disease and controls. Lab Invest (1987) 1.73

Neurofilament-deficient axons and perikaryal aggregates in viable transgenic mice expressing a neurofilament-beta-galactosidase fusion protein. Neuron (1994) 1.69

Neurofibrillary axonal swellings and amyotrophic lateral sclerosis. J Neurol Sci (1984) 1.65

Down's syndrome: abnormal neuromuscular junction in tongue of transgenic mice with elevated levels of human Cu/Zn-superoxide dismutase. Cell (1988) 1.62

Assembly of type IV neuronal intermediate filaments in nonneuronal cells in the absence of preexisting cytoplasmic intermediate filaments. J Cell Biol (1993) 1.61

Mutations associated with amyotrophic lateral sclerosis convert superoxide dismutase from an antiapoptotic gene to a proapoptotic gene: studies in yeast and neural cells. Proc Natl Acad Sci U S A (1995) 1.51

A mutant neurofilament subunit causes massive, selective motor neuron death: implications for the pathogenesis of human motor neuron disease. Neuron (1994) 1.46

Ubiquitin and microtubule-associated protein tau immunoreactivity each define distinct structures with differing distributions and solubility properties in Alzheimer brain. Proc Natl Acad Sci U S A (1988) 1.41

The molecular biology of intermediate filament proteins. Int Rev Cytol (1992) 1.40

Phosphorylation of neurofilaments is altered in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol (1988) 1.40

Monoclonal antibodies to gel-excised glial filament protein and their reactivities with other intermediate filament proteins. J Neurochem (1984) 1.33

Alpha-internexin, a novel neuronal intermediate filament protein, precedes the low molecular weight neurofilament protein (NF-L) in the developing rat brain. J Neurosci (1990) 1.29

Cytopathology of amyotrophic lateral sclerosis. Adv Neurol (1991) 1.28

Cu/Zn superoxide dismutase activity in familial and sporadic amyotrophic lateral sclerosis. J Neurochem (1994) 1.25

SOD1 mutation is associated with accumulation of neurofilaments in amyotrophic lateral sclerosis. Ann Neurol (1996) 1.19

Phosphorylated high molecular weight neurofilament protein in lower motor neurons in amyotrophic lateral sclerosis and other neurodegenerative diseases involving ventral horn cells. Acta Neuropathol (1990) 1.19

Attenuation of methamphetamine-induced neurotoxicity in copper/zinc superoxide dismutase transgenic mice. J Neurochem (1994) 1.14

Interaction of the tail domain of high molecular weight subunits of neurofilaments with the COOH-terminal region of tubulin and its regulation by tau protein kinase II. J Biol Chem (1993) 1.13

Peripherin and neurofilament protein coexist in spinal spheroids of motor neuron disease. J Neuropathol Exp Neurol (1992) 1.07

Ubiquitin in neurodegenerative diseases. Brain Pathol (1993) 1.03

Comparison of sporadic and familial disease amongst 580 cases of motor neuron disease. J Neurol Neurosurg Psychiatry (1988) 0.99

Cu/Zn superoxide dismutase mRNA and enzyme activity, and susceptibility to lipid peroxidation, increases with aging in murine brains. Brain Res Mol Brain Res (1992) 0.96

Down's syndrome: morphological remodelling and increased complexity in the neuromuscular junction of transgenic CuZn-superoxide dismutase mice. J Neurocytol (1991) 0.96

Peripherin immunoreactive structures in amyotrophic lateral sclerosis. Lab Invest (1993) 0.93

Some neural intermediate filaments contain both peripherin and the neurofilament proteins. J Neurosci Res (1991) 0.93

Electrophysiologic studies in the diagnosis and prognosis of motor neuron diseases. Neurol Clin (1985) 0.91

Molecular phenotype of simian virus 40 large T antigen-induced primitive neuroectodermal tumors in four different lines of transgenic mice. Lab Invest (1994) 0.91

Reactive astrocytes are widespread in the cortical gray matter of amyotrophic lateral sclerosis. J Neurosci Res (1994) 0.86

Copper/zinc superoxide dismutase mRNA levels are increased in sporadic amyotrophic lateral sclerosis motorneurons. Brain Res (1994) 0.82

Ubiquitin and neurofilament expression in anterior horn cells in amyotrophic lateral sclerosis: possible clues to the pathogenesis. Neuropathol Appl Neurobiol (1994) 0.82

Ultrastructural study of skein-like inclusions in anterior horn neurons of patients with motor neuron disease. Neurosci Lett (1992) 0.81

Hippocampal mossy fiber changes in mice transgenic for the human copper-zinc superoxide dismutase gene. Neurosci Lett (1993) 0.79

Articles by these authors

Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium. Neurology (2005) 21.67

Alpha-synuclein in Lewy bodies. Nature (1997) 20.83

Clinical and pathological diagnosis of frontotemporal dementia: report of the Work Group on Frontotemporal Dementia and Pick's Disease. Arch Neurol (2001) 9.96

Neurodegenerative tauopathies. Annu Rev Neurosci (2001) 9.53

Transplantation of embryonic dopamine neurons for severe Parkinson's disease. N Engl J Med (2001) 9.51

Second consensus statement on the diagnosis of multiple system atrophy. Neurology (2008) 7.27

Alzheimer's Disease Neuroimaging Initiative (ADNI): clinical characterization. Neurology (2009) 7.04

Consensus recommendations for the postmortem diagnosis of Alzheimer disease from the National Institute on Aging and the Reagan Institute Working Group on diagnostic criteria for the neuropathological assessment of Alzheimer disease. J Neuropathol Exp Neurol (1997) 6.12

Comparing predictors of conversion and decline in mild cognitive impairment. Neurology (2010) 5.69

Oxidative damage linked to neurodegeneration by selective alpha-synuclein nitration in synucleinopathy lesions. Science (2000) 5.62

A68: a major subunit of paired helical filaments and derivatized forms of normal Tau. Science (1991) 5.60

Membrane-anchored aspartyl protease with Alzheimer's disease beta-secretase activity. Nature (1999) 5.48

Aggregation of alpha-synuclein in Lewy bodies of sporadic Parkinson's disease and dementia with Lewy bodies. Am J Pathol (1998) 5.47

MRI of hippocampal volume loss in early Alzheimer's disease in relation to ApoE genotype and biomarkers. Brain (2009) 4.51

Relationships between biomarkers in aging and dementia. Neurology (2009) 4.16

Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol (1996) 4.10

Mutation-specific functional impairments in distinct tau isoforms of hereditary FTDP-17. Science (1998) 4.07

A hydrophobic stretch of 12 amino acid residues in the middle of alpha-synuclein is essential for filament assembly. J Biol Chem (2000) 3.97

Synucleins are developmentally expressed, and alpha-synuclein regulates the size of the presynaptic vesicular pool in primary hippocampal neurons. J Neurosci (2000) 3.90

Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron (1997) 3.73

MRI and CSF biomarkers in normal, MCI, and AD subjects: predicting future clinical change. Neurology (2009) 3.72

Age-dependent emergence and progression of a tauopathy in transgenic mice overexpressing the shortest human tau isoform. Neuron (1999) 3.49

Abnormal tau phosphorylation at Ser396 in Alzheimer's disease recapitulates development and contributes to reduced microtubule binding. Neuron (1993) 3.45

Epitopes that span the tau molecule are shared with paired helical filaments. Neuron (1988) 3.28

Monoclonal antibody PHF-1 recognizes tau protein phosphorylated at serine residues 396 and 404. J Neurosci Res (1994) 3.26

Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2000) 3.11

Operational definition of normal sinus heart rate. Am J Cardiol (1992) 3.08

Second conserved domain of gp120 is important for HIV infectivity and antibody neutralization. Science (1988) 3.08

Activation of the Wnt signaling pathway: a molecular mechanism for lithium action. Dev Biol (1997) 3.03

Increased lipid peroxidation precedes amyloid plaque formation in an animal model of Alzheimer amyloidosis. J Neurosci (2001) 2.89

DLB and PDD boundary issues: diagnosis, treatment, molecular pathology, and biomarkers. Neurology (2007) 2.86

MRI and CSF biomarkers in normal, MCI, and AD subjects: diagnostic discrimination and cognitive correlations. Neurology (2009) 2.83

Relationship of microglial and astrocytic activation to disease onset and progression in a transgenic model of familial ALS. Glia (1998) 2.82

Local modulation of neurofilament phosphorylation, axonal caliber, and slow axonal transport by myelinating Schwann cells. Cell (1992) 2.80

The levels of soluble versus insoluble brain Abeta distinguish Alzheimer's disease from normal and pathologic aging. Exp Neurol (1999) 2.73

The human mid-size neurofilament subunit: a repeated protein sequence and the relationship of its gene to the intermediate filament gene family. EMBO J (1987) 2.72

CSF amyloid {beta} 1-42 predicts cognitive decline in Parkinson disease. Neurology (2010) 2.67

Glucocerebrosidase mutations are an important risk factor for Lewy body disorders. Neurology (2006) 2.65

Amyloid vs FDG-PET in the differential diagnosis of AD and FTLD. Neurology (2011) 2.61

Genetic evidence for the involvement of tau in progressive supranuclear palsy. Ann Neurol (1997) 2.60

Reactive oxygen species, cell signaling, and cell injury. Free Radic Biol Med (2000) 2.52

Pure, postmitotic, polarized human neurons derived from NTera 2 cells provide a system for expressing exogenous proteins in terminally differentiated neurons. J Neurosci (1992) 2.50

Glial cytoplasmic inclusions in white matter oligodendrocytes of multiple system atrophy brains contain insoluble alpha-synuclein. Ann Neurol (1998) 2.47

Myelin-associated glycoprotein is a myelin signal that modulates the caliber of myelinated axons. J Neurosci (1998) 2.43

Maturation and endosomal targeting of beta-site amyloid precursor protein-cleaving enzyme. The Alzheimer's disease beta-secretase. J Biol Chem (2000) 2.42

Synucleinopathies: clinical and pathological implications. Arch Neurol (2001) 2.36

Nestin expression in embryonic human neuroepithelium and in human neuroepithelial tumor cells. Lab Invest (1992) 2.30

Axon pathology in Parkinson's disease and Lewy body dementia hippocampus contains alpha-, beta-, and gamma-synuclein. Proc Natl Acad Sci U S A (1999) 2.30

Predictors of extubation outcome in patients who have successfully completed a spontaneous breathing trial. Chest (2001) 2.29

Alpha-synuclein cortical Lewy bodies correlate with dementia in Parkinson's disease. Neurology (2000) 2.27

Missense and silent tau gene mutations cause frontotemporal dementia with parkinsonism-chromosome 17 type, by affecting multiple alternative RNA splicing regulatory elements. Proc Natl Acad Sci U S A (1999) 2.26

Monoclonal antibodies distinguish several differentially phosphorylated states of the two largest rat neurofilament subunits (NF-H and NF-M) and demonstrate their existence in the normal nervous system of adult rats. J Neurosci (1987) 2.26

Clinical and neuropathologic variation in neuronal intermediate filament inclusion disease. Neurology (2004) 2.23

Serial MRI and CSF biomarkers in normal aging, MCI, and AD. Neurology (2010) 2.20

Induction of alpha-synuclein aggregation by intracellular nitrative insult. J Neurosci (2001) 2.16

Alzheimer's A beta(1-42) is generated in the endoplasmic reticulum/intermediate compartment of NT2N cells. Nat Med (1997) 2.15

Two-stage expression of neurofilament polypeptides during rat neurogenesis with early establishment of adult phosphorylation patterns. J Neurosci (1987) 2.15

Mutant and wild type human alpha-synucleins assemble into elongated filaments with distinct morphologies in vitro. J Biol Chem (1999) 2.13

Biopsy-derived adult human brain tau is phosphorylated at many of the same sites as Alzheimer's disease paired helical filament tau. Neuron (1994) 2.13

Pathogenic implications of mutations in the tau gene in pallido-ponto-nigral degeneration and related neurodegenerative disorders linked to chromosome 17. Proc Natl Acad Sci U S A (1998) 2.13

Lithium reduces tau phosphorylation by inhibition of glycogen synthase kinase-3. J Biol Chem (1997) 2.12

Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis. Am J Pathol (1994) 2.12

Frontotemporal dementia progresses to death faster than Alzheimer disease. Neurology (2005) 2.10

Cognitive and motor assessment in autopsy-proven corticobasal degeneration. Neurology (2007) 2.09

Dityrosine cross-linking promotes formation of stable alpha -synuclein polymers. Implication of nitrative and oxidative stress in the pathogenesis of neurodegenerative synucleinopathies. J Biol Chem (2000) 2.02

Distinct MRI atrophy patterns in autopsy-proven Alzheimer's disease and frontotemporal lobar degeneration. Am J Alzheimers Dis Other Demen (2008) 2.00

Pathological changes in olfactory neurons in patients with Alzheimer's disease. Nature (1989) 2.00

Differences in cell-to-cell spread of pathogenic and apathogenic rabies virus in vivo and in vitro. J Virol (1985) 2.00

The abnormal phosphorylation of tau protein at Ser-202 in Alzheimer disease recapitulates phosphorylation during development. Proc Natl Acad Sci U S A (1993) 1.99

Autosomal dominant dementia with widespread neurofibrillary tangles. Ann Neurol (1997) 1.98

A panel of epitope-specific antibodies detects protein domains distributed throughout human alpha-synuclein in Lewy bodies of Parkinson's disease. J Neurosci Res (2000) 1.97

Neuropathological changes in two lines of mice carrying a transgene for mutant human Cu,Zn SOD, and in mice overexpressing wild type human SOD: a model of familial amyotrophic lateral sclerosis (FALS). Brain Res (1995) 1.97

NTera 2 cells: a human cell line which displays characteristics expected of a human committed neuronal progenitor cell. J Neurosci Res (1993) 1.97

Neurofilaments and orthograde transport are reduced in ventral root axons of transgenic mice that express human SOD1 with a G93A mutation. J Cell Biol (1997) 1.95

Lewy bodies contain altered alpha-synuclein in brains of many familial Alzheimer's disease patients with mutations in presenilin and amyloid precursor protein genes. Am J Pathol (1998) 1.93

Mechanism of glibenclamide inhibition of cystic fibrosis transmembrane conductance regulator Cl- channels expressed in a murine cell line. J Physiol (1997) 1.92