Published in Mol Cell Neurosci on June 01, 2004
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Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis. Nat Rev Neurol (2011) 2.73
Genetic evidence implicates the immune system and cholesterol metabolism in the aetiology of Alzheimer's disease. PLoS One (2010) 2.57
Mitochondrial function and actin regulate dynamin-related protein 1-dependent mitochondrial fission. Curr Biol (2005) 2.54
Antiviral protein APOBEC3G localizes to ribonucleoprotein complexes found in P bodies and stress granules. J Virol (2006) 2.50
The serine protease Omi/HtrA2 regulates apoptosis by binding XIAP through a reaper-like motif. J Biol Chem (2001) 2.47
Tau phosphorylation: the therapeutic challenge for neurodegenerative disease. Trends Mol Med (2009) 2.39
Biomarkers in amyotrophic lateral sclerosis. Lancet Neurol (2009) 2.20
An MND/ALS phenotype associated with C9orf72 repeat expansion: abundant p62-positive, TDP-43-negative inclusions in cerebral cortex, hippocampus and cerebellum but without associated cognitive decline. Neuropathology (2011) 2.16
Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability. Proc Natl Acad Sci U S A (2012) 2.14
Is language impairment more common than executive dysfunction in amyotrophic lateral sclerosis? J Neurol Neurosurg Psychiatry (2012) 2.07
Exclusively targeting beta-secretase to lipid rafts by GPI-anchor addition up-regulates beta-site processing of the amyloid precursor protein. Proc Natl Acad Sci U S A (2003) 2.03
Reduced expression of the Kinesin-Associated Protein 3 (KIFAP3) gene increases survival in sporadic amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2009) 2.03
Systemic delivery of scAAV9 expressing SMN prolongs survival in a model of spinal muscular atrophy. Sci Transl Med (2010) 2.02
Caspase-6 gene disruption reveals a requirement for lamin A cleavage in apoptotic chromatin condensation. EMBO J (2002) 1.82
Intraventricular enzyme replacement improves disease phenotypes in a mouse model of late infantile neuronal ceroid lipofuscinosis. Mol Ther (2008) 1.82
Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis. Neurobiol Dis (2004) 1.75
Tumor necrosis factor-alpha convertase (ADAM17) mediates regulated ectodomain shedding of the severe-acute respiratory syndrome-coronavirus (SARS-CoV) receptor, angiotensin-converting enzyme-2 (ACE2). J Biol Chem (2005) 1.73
Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: the NNIPPS study. Brain (2008) 1.72
The C9ORF72 expansion mutation is a common cause of ALS+/-FTD in Europe and has a single founder. Eur J Hum Genet (2012) 1.71
Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis. Neurobiol Dis (2006) 1.69
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Low index-to-ring finger length ratio in sporadic ALS supports prenatally defined motor neuronal vulnerability. J Neurol Neurosurg Psychiatry (2011) 1.67
CNS-directed AAV2-mediated gene therapy ameliorates functional deficits in a murine model of infantile neuronal ceroid lipofuscinosis. Mol Ther (2005) 1.66
Neuronal membrane cholesterol loss enhances amyloid peptide generation. J Cell Biol (2004) 1.66
Amyotrophic lateral sclerosis in an urban setting: a population based study of inner city London. J Neurol (2006) 1.61
Direct evidence for axonal transport defects in a novel mouse model of mutant spastin-induced hereditary spastic paraplegia (HSP) and human HSP patients. J Neurochem (2009) 1.61
Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates. J Proteome Res (2007) 1.59
Contrasting effects of haloperidol and lithium on rodent brain structure: a magnetic resonance imaging study with postmortem confirmation. Biol Psychiatry (2012) 1.56
Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain. Sci Rep (2012) 1.54
BACE1 (beta-secretase) transgenic and knockout mice: identification of neurochemical deficits and behavioral changes. Mol Cell Neurosci (2003) 1.52
The FE65 proteins and Alzheimer's disease. J Neurosci Res (2008) 1.51
Dominant mutations in the cation channel gene transient receptor potential vanilloid 4 cause an unusual spectrum of neuropathies. Brain (2010) 1.51
FUS-SMN protein interactions link the motor neuron diseases ALS and SMA. Cell Rep (2012) 1.50
Neurofilaments and neurological disease. Bioessays (2003) 1.50
Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy. Proc Natl Acad Sci U S A (2013) 1.49
ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43. Proc Natl Acad Sci U S A (2013) 1.48
Synaptic NMDA receptor activation stimulates alpha-secretase amyloid precursor protein processing and inhibits amyloid-beta production. J Neurosci (2009) 1.47
Cdk5/p35 phosphorylates lemur tyrosine kinase-2 to regulate protein phosphatase-1C phosphorylation and activity. J Neurochem (2012) 1.47
Biguanide metformin acts on tau phosphorylation via mTOR/protein phosphatase 2A (PP2A) signaling. Proc Natl Acad Sci U S A (2010) 1.46
Variants of the elongator protein 3 (ELP3) gene are associated with motor neuron degeneration. Hum Mol Genet (2008) 1.46
Rapid tyrosine phosphorylation of neuronal proteins including tau and focal adhesion kinase in response to amyloid-beta peptide exposure: involvement of Src family protein kinases. J Neurosci (2002) 1.46
Functional magnetic resonance imaging of verbal fluency and confrontation naming using compressed image acquisition to permit overt responses. Hum Brain Mapp (2003) 1.38
Phosphorylation regulates tau interactions with Src homology 3 domains of phosphatidylinositol 3-kinase, phospholipase Cgamma1, Grb2, and Src family kinases. J Biol Chem (2008) 1.38
Comparison of cellular ribonucleoprotein complexes associated with the APOBEC3F and APOBEC3G antiviral proteins. J Virol (2008) 1.37
Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion. Acta Neuropathol (2012) 1.36
Parkinson's disease alpha-synuclein mutations exhibit defective axonal transport in cultured neurons. J Cell Sci (2004) 1.36
Tyrosine 394 is phosphorylated in Alzheimer's paired helical filament tau and in fetal tau with c-Abl as the candidate tyrosine kinase. J Neurosci (2005) 1.35
Analysis of alternative splicing associated with aging and neurodegeneration in the human brain. Genome Res (2011) 1.35
Late onset neurodegeneration in the Cln3-/- mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation. Brain Res (2004) 1.32
AddNeuroMed--the European collaboration for the discovery of novel biomarkers for Alzheimer's disease. Ann N Y Acad Sci (2009) 1.30
Clinical care of patients with amyotrophic lateral sclerosis. Lancet Neurol (2007) 1.30
Two families with familial amyotrophic lateral sclerosis are linked to a novel locus on chromosome 16q. Am J Hum Genet (2003) 1.29
Isolation and characterisation of potential respiratory syncytial virus receptor(s) on epithelial cells. Microbes Infect (2003) 1.28
Mutations in CHMP2B in lower motor neuron predominant amyotrophic lateral sclerosis (ALS). PLoS One (2010) 1.28
Effects of cis-regulatory variation differ across regions of the adult human brain. Hum Mol Genet (2010) 1.27
The neuronal adaptor protein X11alpha reduces Abeta levels in the brains of Alzheimer's APPswe Tg2576 transgenic mice. J Biol Chem (2003) 1.27
High resolution 1H NMR-based metabolomics indicates a neurotransmitter cycling deficit in cerebral tissue from a mouse model of Batten disease. J Biol Chem (2005) 1.27
Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease. Hum Mol Genet (2009) 1.26
Detection, quantitation, purification, and identification of cardiac proteins S-thiolated during ischemia and reperfusion. J Biol Chem (2002) 1.25
Neuroprotection of host cells by human central nervous system stem cells in a mouse model of infantile neuronal ceroid lipofuscinosis. Cell Stem Cell (2009) 1.25
An autoantibody inhibitory to glutamic acid decarboxylase in the neurodegenerative disorder Batten disease. Hum Mol Genet (2002) 1.24
Membrane-bound beta-amyloid oligomers are recruited into lipid rafts by a fyn-dependent mechanism. FASEB J (2007) 1.24
Neurofilament heavy chain side arm phosphorylation regulates axonal transport of neurofilaments. J Cell Biol (2003) 1.24