Published in Ann Neurol on August 01, 2003
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Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science (2006) 27.96
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Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations. Ann Neurol (2007) 6.58
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The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS. Science (2013) 6.34
CSF markers for incipient Alzheimer's disease. Lancet Neurol (2003) 6.12
Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions. Nat Genet (2010) 5.52
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A mutation in VPS35, encoding a subunit of the retromer complex, causes late-onset Parkinson disease. Am J Hum Genet (2011) 4.52
A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain (2009) 4.40
Pathological heterogeneity of frontotemporal lobar degeneration with ubiquitin-positive inclusions delineated by ubiquitin immunohistochemistry and novel monoclonal antibodies. Am J Pathol (2006) 4.32
A harmonized classification system for FTLD-TDP pathology. Acta Neuropathol (2011) 4.15
CSF phosphorylated tau protein correlates with neocortical neurofibrillary pathology in Alzheimer's disease. Brain (2006) 3.95
TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia. Lancet Neurol (2010) 3.92
Erythropoietin therapy for acute stroke is both safe and beneficial. Mol Med (2002) 3.87
Prevalence and prognostic value of CSF markers of Alzheimer's disease pathology in patients with subjective cognitive impairment or mild cognitive impairment in the DESCRIPA study: a prospective cohort study. Lancet Neurol (2009) 3.65
ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import. EMBO J (2010) 3.58
Presenilin clinical mutations can affect gamma-secretase activity by different mechanisms. J Neurochem (2006) 3.03
TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations. J Neuropathol Exp Neurol (2007) 2.97
Biological markers for therapeutic trials in Alzheimer's disease. Proceedings of the biological markers working group; NIA initiative on neuroimaging in Alzheimer's disease. Neurobiol Aging (2003) 2.96
Concomitant TAR-DNA-binding protein 43 pathology is present in Alzheimer disease and corticobasal degeneration but not in other tauopathies. J Neuropathol Exp Neurol (2008) 2.96
Cerebral amyloid-β PET with florbetaben (18F) in patients with Alzheimer's disease and healthy controls: a multicentre phase 2 diagnostic study. Lancet Neurol (2011) 2.84
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies. Acta Neuropathol (2009) 2.76
Mutations in the colony stimulating factor 1 receptor (CSF1R) gene cause hereditary diffuse leukoencephalopathy with spheroids. Nat Genet (2011) 2.76
The mechanism of γ-Secretase dysfunction in familial Alzheimer disease. EMBO J (2012) 2.72
Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease. Acta Neuropathol (2009) 2.68
Clinical and pathological continuum of multisystem TDP-43 proteinopathies. Arch Neurol (2009) 2.68
Genetic prion disease: the EUROCJD experience. Hum Genet (2005) 2.64
Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies. J Biol Chem (2009) 2.60
Genetic evidence implicates the immune system and cholesterol metabolism in the aetiology of Alzheimer's disease. PLoS One (2010) 2.57
FUS pathology in basophilic inclusion body disease. Acta Neuropathol (2009) 2.52
Advances in understanding the molecular basis of frontotemporal dementia. Nat Rev Neurol (2012) 2.48
Unrecognized vitamin D3 deficiency is common in Parkinson disease: Harvard Biomarker Study. Neurology (2013) 2.47
Misfolded proteinase K-resistant hyperphosphorylated alpha-synuclein in aged transgenic mice with locomotor deterioration and in human alpha-synucleinopathies. J Clin Invest (2002) 2.47
The synthetic cannabinoid Spice as a trigger for an acute exacerbation of cannabis induced recurrent psychotic episodes. Schizophr Res (2010) 2.45
gamma-Secretase heterogeneity in the Aph1 subunit: relevance for Alzheimer's disease. Science (2009) 2.43
Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Am J Pathol (2008) 2.41
Knockdown of transactive response DNA-binding protein (TDP-43) downregulates histone deacetylase 6. EMBO J (2009) 2.37
Frontotemporal lobar degeneration: demographic characteristics of 353 patients. Arch Neurol (2005) 2.36
Focal decline of cortical thickness in Alzheimer's disease identified by computational neuroanatomy. Cereb Cortex (2004) 2.36
Direct quantification of CSF alpha-synuclein by ELISA and first cross-sectional study in patients with neurodegeneration. Exp Neurol (2008) 2.31
FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration. Acta Neuropathol (2010) 2.30
hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations. Acta Neuropathol (2013) 2.27
Prediction of conversion from mild cognitive impairment to Alzheimer's disease dementia based upon biomarkers and neuropsychological test performance. Neurobiol Aging (2010) 2.27
MDS Task Force on mild cognitive impairment in Parkinson's disease: critical review of PD-MCI. Mov Disord (2011) 2.27
The spectrum of mutations in progranulin: a collaborative study screening 545 cases of neurodegeneration. Arch Neurol (2010) 2.19
GATA transcription factors directly regulate the Parkinson's disease-linked gene alpha-synuclein. Proc Natl Acad Sci U S A (2008) 2.17
Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathol (2011) 2.17
Associated factors for REM sleep behavior disorder in Parkinson disease. Neurology (2011) 2.17
ALDH1A1 is a marker of astrocytic differentiation during brain development and correlates with better survival in glioblastoma patients. Brain Pathol (2012) 2.14
TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease. Acta Neuropathol (2007) 2.12
Automated cortical thickness measurements from MRI can accurately separate Alzheimer's patients from normal elderly controls. Neurobiol Aging (2006) 2.09
Measurement of phosphorylated tau epitopes in the differential diagnosis of Alzheimer disease: a comparative cerebrospinal fluid study. Arch Gen Psychiatry (2004) 2.07
Socioeconomic status and depression during and after pregnancy in the Franconian Maternal Health Evaluation Studies (FRAMES). Arch Gynecol Obstet (2013) 2.06
FieF (YiiP) from Escherichia coli mediates decreased cellular accumulation of iron and relieves iron stress. Arch Microbiol (2004) 2.06
TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97. J Neurosci (2010) 2.05
Multivariate deformation-based analysis of brain atrophy to predict Alzheimer's disease in mild cognitive impairment. Neuroimage (2007) 2.03
Measurement of basal forebrain atrophy in Alzheimer's disease using MRI. Brain (2005) 2.03
TDP-43-negative FTLD-U is a significant new clinico-pathological subtype of FTLD. Acta Neuropathol (2008) 2.02
MicroRNAs in cerebrospinal fluid identify glioblastoma and metastatic brain cancers and reflect disease activity. Neuro Oncol (2012) 2.02
TDP-43-positive white matter pathology in frontotemporal lobar degeneration with ubiquitin-positive inclusions. J Neuropathol Exp Neurol (2007) 2.02
Measurement of alpha- and beta-secretase cleaved amyloid precursor protein in cerebrospinal fluid from Alzheimer patients. Exp Neurol (2003) 1.99
Synapse formation and function is modulated by the amyloid precursor protein. J Neurosci (2006) 1.99
Designing drug trials for Alzheimer's disease: what we have learned from the release of the phase III antibody trials: a report from the EU/US/CTAD Task Force. Alzheimers Dement (2013) 1.98
Hyperphosphorylation and insolubility of alpha-synuclein in transgenic mouse oligodendrocytes. EMBO Rep (2002) 1.91
Viable c-Kit(W/W) mutants reveal pivotal role for c-kit in the maintenance of lymphopoiesis. Immunity (2002) 1.87
Missense mutations in the progranulin gene linked to frontotemporal lobar degeneration with ubiquitin-immunoreactive inclusions reduce progranulin production and secretion. J Biol Chem (2007) 1.87
The Alzheimer's Association external quality control program for cerebrospinal fluid biomarkers. Alzheimers Dement (2011) 1.87
Standardization of preanalytical aspects of cerebrospinal fluid biomarker testing for Alzheimer's disease diagnosis: a consensus paper from the Alzheimer's Biomarkers Standardization Initiative. Alzheimers Dement (2011) 1.85
Intravenous immunoglobulin for treatment of mild-to-moderate Alzheimer's disease: a phase 2, randomised, double-blind, placebo-controlled, dose-finding trial. Lancet Neurol (2013) 1.84
Association of cerebrospinal fluid β-amyloid 1-42, T-tau, P-tau181, and α-synuclein levels with clinical features of drug-naive patients with early Parkinson disease. JAMA Neurol (2013) 1.83
Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins. Acta Neuropathol (2013) 1.82
The cellular prion protein mediates neurotoxic signalling of β-sheet-rich conformers independent of prion replication. EMBO J (2011) 1.80
Two German kindreds with familial amyotrophic lateral sclerosis due to TARDBP mutations. Arch Neurol (2008) 1.78