Published in Rinsho Shinkeigaku on November 01, 2010
TDP-43 mutation in familial amyotrophic lateral sclerosis. Ann Neurol (2008) 2.92
Negative regulation of the RIG-I signaling by the ubiquitin ligase RNF125. Proc Natl Acad Sci U S A (2007) 2.88
Prospective 10-year surveillance of human prion diseases in Japan. Brain (2010) 2.41
Efficacy and safety of leuprorelin in patients with spinal and bulbar muscular atrophy (JASMITT study): a multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol (2010) 2.33
Three different neutrophil subsets exhibited in mice with different susceptibilities to infection by methicillin-resistant Staphylococcus aureus. Immunity (2004) 2.29
Axonal alpha-synuclein aggregates herald centripetal degeneration of cardiac sympathetic nerve in Parkinson's disease. Brain (2007) 2.10
TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation. Acta Neuropathol (2007) 2.01
Association of HTRA1 mutations and familial ischemic cerebral small-vessel disease. N Engl J Med (2009) 1.96
CAG repeat disorder models and human neuropathology: similarities and differences. Acta Neuropathol (2007) 1.86
Loss of poly(ADP-ribose) glycohydrolase causes progressive neurodegeneration in Drosophila melanogaster. Proc Natl Acad Sci U S A (2003) 1.85
Haploinsufficiency of CSF-1R and clinicopathologic characterization in patients with HDLS. Neurology (2013) 1.81
Hepatitis C virus non-structural proteins in the probable membranous compartment function in viral genome replication. J Biol Chem (2003) 1.77
Mutations in SPG11 are frequent in autosomal recessive spastic paraplegia with thin corpus callosum, cognitive decline and lower motor neuron degeneration. Brain (2007) 1.76
SCA17 homozygote showing Huntington's disease-like phenotype. Ann Neurol (2004) 1.72
Phosphorylated alpha-synuclein is ubiquitinated in alpha-synucleinopathy lesions. J Biol Chem (2002) 1.71
Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic. Hum Mol Genet (2007) 1.66
Differential levels of alpha-synuclein, beta-amyloid42 and tau in CSF between patients with dementia with Lewy bodies and Alzheimer's disease. J Neurol Neurosurg Psychiatry (2010) 1.65
Aprataxin, a novel protein that protects against genotoxic stress. Hum Mol Genet (2004) 1.60
Molecular pathology and genetic advances in amyotrophic lateral sclerosis: an emerging molecular pathway and the significance of glial pathology. Acta Neuropathol (2011) 1.58
Baculovirus induces an innate immune response and confers protection from lethal influenza virus infection in mice. J Immunol (2003) 1.57
A mutation in the immunoproteasome subunit PSMB8 causes autoinflammation and lipodystrophy in humans. J Clin Invest (2011) 1.57
Isolation and characterization of patient-derived, toxic, high mass amyloid beta-protein (Abeta) assembly from Alzheimer disease brains. J Biol Chem (2009) 1.52
High frequency of calcification in basal ganglia on brain computed tomography images in Japanese older adults. Geriatr Gerontol Int (2012) 1.49
Degeneration of cardiac sympathetic nerve can occur in multiple system atrophy. Acta Neuropathol (2006) 1.47
Degeneration of cardiac sympathetic nerve begins in the early disease process of Parkinson's disease. Brain Pathol (2007) 1.44
Global democratic consensus on neuropathological disease criteria. Lancet Neurol (2002) 1.44
Transcriptional repression of p53 by parkin and impairment by mutations associated with autosomal recessive juvenile Parkinson's disease. Nat Cell Biol (2009) 1.41
Maturation process of TDP-43-positive neuronal cytoplasmic inclusions in amyotrophic lateral sclerosis with and without dementia. Acta Neuropathol (2008) 1.41
Dropped head syndrome in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener (2012) 1.40
Sporadic amyotrophic lateral sclerosis: two pathological patterns shown by analysis of distribution of TDP-43-immunoreactive neuronal and glial cytoplasmic inclusions. Acta Neuropathol (2008) 1.39
Botulinum toxin A injections improve apraxia of eyelid opening without overt blepharospasm associated with neurodegenerative diseases. Mov Disord (2007) 1.38
Neuropathology with clinical correlations of sporadic amyotrophic lateral sclerosis: 102 autopsy cases examined between 1962 and 2000. Brain Pathol (2003) 1.36
The Lewy body in Parkinson's disease: molecules implicated in the formation and degradation of alpha-synuclein aggregates. Neuropathology (2007) 1.36
Influence of the serotonin transporter gene-linked polymorphic region on the antidepressant response to fluvoxamine in Japanese depressed patients. Prog Neuropsychopharmacol Biol Psychiatry (2002) 1.36
Clinical features of neuromyelitis optica in a large Japanese cohort: comparison between phenotypes. J Neurol Neurosurg Psychiatry (2011) 1.32
Patients homozygous and heterozygous for SNCA duplication in a family with parkinsonism and dementia. Arch Neurol (2008) 1.32
Multiplex families with multiple system atrophy. Arch Neurol (2007) 1.32
Dynamin-binding protein gene on chromosome 10q is associated with late-onset Alzheimer's disease. Hum Mol Genet (2006) 1.27
Symptomatic narcolepsy in patients with neuromyelitis optica and multiple sclerosis: new neurochemical and immunological implications. Arch Neurol (2009) 1.27
Polyglutamine diseases: where does toxicity come from? what is toxicity? where are we going? J Mol Cell Biol (2010) 1.25
Cellular pathology in multiple system atrophy. Neuropathology (2006) 1.25
Selective reduction of a PDZ protein, SAP-97, in the prefrontal cortex of patients with chronic schizophrenia. J Neurochem (2002) 1.24
SORL1 is genetically associated with late-onset Alzheimer's disease in Japanese, Koreans and Caucasians. PLoS One (2013) 1.23
Sporadic ataxias in Japan--a population-based epidemiological study. Cerebellum (2008) 1.22
Cerebellar involvement in progressive supranuclear palsy: A clinicopathological study. Mov Disord (2009) 1.22
Cardiac sympathetic denervation precedes neuronal loss in the sympathetic ganglia in Lewy body disease. Acta Neuropathol (2005) 1.21
Clinicopathological study of diffuse type brainstem gliomas: analysis of 40 autopsy cases. Neurol Med Chir (Tokyo) (2003) 1.21
Gain- and loss-of-function in chick embryos by electroporation. Mech Dev (2004) 1.20
Characterization of causative allergens for wheat-dependent exercise-induced anaphylaxis sensitized with hydrolyzed wheat proteins in facial soap. Allergol Int (2013) 1.20
Subgenomic replicon derived from a cell line infected with the hepatitis C virus. Biochem Biophys Res Commun (2002) 1.19
Demonstration of alpha-synuclein immunoreactivity in neuronal and glial cytoplasm in normal human brain tissue using proteinase K and formic acid pretreatment. Exp Neurol (2002) 1.19
TDP-43-immunoreactive neuronal and glial inclusions in the neostriatum in amyotrophic lateral sclerosis with and without dementia. Acta Neuropathol (2007) 1.18
Genetic association of CTNNA3 with late-onset Alzheimer's disease in females. Hum Mol Genet (2007) 1.15
Cerebellar ataxia rehabilitation trial in degenerative cerebellar diseases. Neurorehabil Neural Repair (2011) 1.14