Published in Acta Neuropathol on July 08, 2008
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. Hum Mol Genet (2010) 5.75
TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci U S A (2010) 3.39
TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity. J Biol Chem (2009) 3.29
Prion-like disorders: blurring the divide between transmissibility and infectivity. J Cell Sci (2010) 2.29
Cell-to-cell transmission of pathogenic proteins in neurodegenerative diseases. Nat Med (2014) 1.85
Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseases. J Neurol (2009) 1.85
Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: a spectrum of TDP-43 proteinopathies. Neuropathology (2010) 1.53
A seeding reaction recapitulates intracellular formation of Sarkosyl-insoluble transactivation response element (TAR) DNA-binding protein-43 inclusions. J Biol Chem (2011) 1.48
TDP-43 in aging and Alzheimer's disease - a review. Int J Clin Exp Pathol (2011) 1.46
Neurodegeneration the RNA way. Prog Neurobiol (2011) 1.43
Staging TDP-43 pathology in Alzheimer's disease. Acta Neuropathol (2013) 1.36
TDP-43 aggregation in neurodegeneration: are stress granules the key? Brain Res (2012) 1.35
Protein aggregation in amyotrophic lateral sclerosis. Acta Neuropathol (2013) 1.32
Extensive FUS-immunoreactive pathology in juvenile amyotrophic lateral sclerosis with basophilic inclusions. Brain Pathol (2010) 1.31
Recent advances in our understanding of neurodegeneration. J Neural Transm (Vienna) (2009) 1.27
Neuropathology of non-Alzheimer degenerative disorders. Int J Clin Exp Pathol (2009) 1.27
Transactivation response DNA-binding protein 43 microvasculopathy in frontotemporal degeneration and familial Lewy body disease. J Neuropathol Exp Neurol (2009) 1.01
TDP-43 skeins show properties of amyloid in a subset of ALS cases. Acta Neuropathol (2012) 0.99
The self-interaction of native TDP-43 C terminus inhibits its degradation and contributes to early proteinopathies. Nat Commun (2012) 0.99
Fine structural analysis of the neuronal inclusions of frontotemporal lobar degeneration with TDP-43 proteinopathy. J Neural Transm (Vienna) (2008) 0.98
Progranulin and TDP-43: mechanistic links and future directions. J Mol Neurosci (2011) 0.90
Drosophila melanogaster as a model organism of brain diseases. Int J Mol Sci (2009) 0.90
TAR DNA-binding protein-43 in amyotrophic lateral sclerosis, frontotemporal lobar degeneration, and Alzheimer disease. Acta Neuropathol (2008) 0.84
Robust cytoplasmic accumulation of phosphorylated TDP-43 in transgenic models of tauopathy. Acta Neuropathol (2013) 0.83
Inhibition of TDP-43 aggregation by nucleic acid binding. PLoS One (2013) 0.83
Casein kinase II induced polymerization of soluble TDP-43 into filaments is inhibited by heat shock proteins. PLoS One (2014) 0.80
TDP-43 inclusion bodies formed in bacteria are structurally amorphous, non-amyloid and inherently toxic to neuroblastoma cells. PLoS One (2014) 0.80
An Amyloid-Like Pathological Conformation of TDP-43 Is Stabilized by Hypercooperative Hydrogen Bonds. Front Mol Neurosci (2016) 0.78
Detection of TDP-43 oligomers in frontotemporal lobar degeneration-TDP. Ann Neurol (2015) 0.77
Point mutations in transactive response DNA-binding protein 43 (TDP-43)'s N-terminal domain compromise its stability, dimerization and functions. J Biol Chem (2017) 0.75
Mutation in the alpha-synuclein gene identified in families with Parkinson's disease. Science (1997) 28.30
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science (2006) 27.96
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem Biophys Res Commun (2006) 10.69
TDP-43 immunoreactivity in hippocampal sclerosis and Alzheimer's disease. Ann Neurol (2007) 5.98
TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions. Am J Pathol (2007) 5.02
Co-morbidity of TDP-43 proteinopathy in Lewy body related diseases. Acta Neuropathol (2007) 3.55
Progranulin mediates caspase-dependent cleavage of TAR DNA binding protein-43. J Neurosci (2007) 3.52
Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Ann Neurol (2008) 3.13
TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor. J Neurochem (2007) 2.91
Concurrence of TDP-43, tau and alpha-synuclein pathology in brains of Alzheimer's disease and dementia with Lewy bodies. Brain Res (2007) 2.38
TDP-43 is deposited in the Guam parkinsonism-dementia complex brains. Brain (2007) 2.25
Colocalization of tau and alpha-synuclein epitopes in Lewy bodies. J Neuropathol Exp Neurol (2003) 2.20
Pathological TDP-43 in parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam. Acta Neuropathol (2007) 1.91
Distinctive neuropathology revealed by alpha-synuclein antibodies in hereditary parkinsonism and dementia linked to chromosome 4p. Acta Neuropathol (2000) 1.79
Filamentous tau in oligodendrocytes and astrocytes of transgenic mice expressing the human tau isoform with the P301L mutation. Am J Pathol (2003) 1.34
Ubiquitin-positive intraneuronal inclusions in the extramotor cortices of presenile dementia patients with motor neuron disease. J Neurol (1992) 1.29
Ubiquitin immunoelectron microscopy of dystrophic neurites in cerebellar senile plaques of Alzheimer's disease. Acta Neuropathol (1990) 1.28
TAR-DNA binding protein 43 in Pick disease. J Neuropathol Exp Neurol (2008) 1.19
Do apoptotic mechanisms regulate synaptic plasticity and growth-cone motility? Neuromolecular Med (2002) 1.18
Ubiquitin-related cytoskeletal abnormality in frontotemporal dementia: immunohistochemical and immunoelectron microscope studies. Acta Neuropathol (1997) 0.95
Ubiquitin-immunohistochemical investigation of atypical Pick's disease without Pick bodies. J Neurol Sci (1998) 0.80
Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron (2011) 20.15
Mutations in LRRK2 cause autosomal-dominant parkinsonism with pleomorphic pathology. Neuron (2004) 14.35
Common variants at MS4A4/MS4A6E, CD2AP, CD33 and EPHA1 are associated with late-onset Alzheimer's disease. Nat Genet (2011) 10.07
Genome-wide analysis of genetic loci associated with Alzheimer disease. JAMA (2010) 9.52
Common variants at ABCA7, MS4A6A/MS4A4E, EPHA1, CD33 and CD2AP are associated with Alzheimer's disease. Nat Genet (2011) 9.23
Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol (2007) 8.10
Clinical diagnostic criteria for dementia associated with Parkinson's disease. Mov Disord (2007) 6.92
Neuropathologic features of amnestic mild cognitive impairment. Arch Neurol (2006) 6.20
National Institute on Aging-Alzheimer's Association guidelines for the neuropathologic assessment of Alzheimer's disease: a practical approach. Acta Neuropathol (2011) 6.00
TDP-43 immunoreactivity in hippocampal sclerosis and Alzheimer's disease. Ann Neurol (2007) 5.98
Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS. Neuron (2013) 5.91
Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions. Nat Genet (2010) 5.52
National Institute on Aging-Alzheimer's Association guidelines for the neuropathologic assessment of Alzheimer's disease. Alzheimers Dement (2012) 5.10
Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol (2009) 4.73
Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations. Acta Neuropathol (2008) 4.73
Neuropathologic outcome of mild cognitive impairment following progression to clinical dementia. Arch Neurol (2006) 4.56
Neuropathology of nondemented aging: presumptive evidence for preclinical Alzheimer disease. Neurobiol Aging (2009) 4.36
Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain (2006) 4.07
Genetic variation in PCDH11X is associated with susceptibility to late-onset Alzheimer's disease. Nat Genet (2009) 4.00
DCTN1 mutations in Perry syndrome. Nat Genet (2009) 3.87
Primary age-related tauopathy (PART): a common pathology associated with human aging. Acta Neuropathol (2014) 3.75
Novel mutations in TARDBP (TDP-43) in patients with familial amyotrophic lateral sclerosis. PLoS Genet (2008) 3.66
Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity. Proc Natl Acad Sci U S A (2009) 3.65
Neuropathologically defined subtypes of Alzheimer's disease with distinct clinical characteristics: a retrospective study. Lancet Neurol (2011) 3.63
Association of LRRK2 exonic variants with susceptibility to Parkinson's disease: a case-control study. Lancet Neurol (2011) 3.36
The high-affinity HSP90-CHIP complex recognizes and selectively degrades phosphorylated tau client proteins. J Clin Invest (2007) 3.35
Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72. Acta Neuropathol (2011) 3.34
Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin and sporadics. Brain (2012) 3.08
Criteria for the diagnosis of corticobasal degeneration. Neurology (2013) 3.07
Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy. Nat Genet (2011) 2.97
A novel human disease with abnormal prion protein sensitive to protease. Ann Neurol (2008) 2.95
Mutations in the colony stimulating factor 1 receptor (CSF1R) gene cause hereditary diffuse leukoencephalopathy with spheroids. Nat Genet (2011) 2.76
Progranulin mutations in primary progressive aphasia: the PPA1 and PPA3 families. Arch Neurol (2007) 2.68
A yeast functional screen predicts new candidate ALS disease genes. Proc Natl Acad Sci U S A (2011) 2.68
Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice. J Neurosci (2010) 2.60
Alzheimer disease with amygdala Lewy bodies: a distinct form of alpha-synucleinopathy. J Neuropathol Exp Neurol (2006) 2.57
Common variation in the miR-659 binding-site of GRN is a major risk factor for TDP43-positive frontotemporal dementia. Hum Mol Genet (2008) 2.48
Abeta40 inhibits amyloid deposition in vivo. J Neurosci (2007) 2.47
Plasma progranulin levels predict progranulin mutation status in frontotemporal dementia patients and asymptomatic family members. Brain (2009) 2.45
Characterization of frontotemporal dementia and/or amyotrophic lateral sclerosis associated with the GGGGCC repeat expansion in C9ORF72. Brain (2012) 2.43
Progranulin in frontotemporal lobar degeneration and neuroinflammation. J Neuroinflammation (2007) 2.43
Risk factors for dementia with Lewy bodies: a case-control study. Neurology (2013) 2.38
FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration. Acta Neuropathol (2010) 2.30
Association between repeat sizes and clinical and pathological characteristics in carriers of C9ORF72 repeat expansions (Xpansize-72): a cross-sectional cohort study. Lancet Neurol (2013) 2.29
Brain expression genome-wide association study (eGWAS) identifies human disease-associated variants. PLoS Genet (2012) 2.23
Accelerated lipofuscinosis and ubiquitination in granulin knockout mice suggest a role for progranulin in successful aging. Am J Pathol (2010) 2.20
Colocalization of tau and alpha-synuclein epitopes in Lewy bodies. J Neuropathol Exp Neurol (2003) 2.20
Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations. J Neuropathol Exp Neurol (2007) 2.19
Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathol (2011) 2.17
Parkinson disease neuropathology: later-developing dementia and loss of the levodopa response. Arch Neurol (2002) 2.17
Neuroimaging correlates of pathologically defined subtypes of Alzheimer's disease: a case-control study. Lancet Neurol (2012) 2.09
Novel late-onset Alzheimer disease loci variants associate with brain gene expression. Neurology (2012) 2.08
Strikingly different clinicopathological phenotypes determined by progranulin-mutation dosage. Am J Hum Genet (2012) 2.07
Age-specific and sex-specific prevalence and incidence of mild cognitive impairment, dementia, and Alzheimer dementia in blacks and whites: a report from the Einstein Aging Study. Alzheimer Dis Assoc Disord (2012) 2.02
Clinically undetected motor neuron disease in pathologically proven frontotemporal lobar degeneration with motor neuron disease. Arch Neurol (2006) 2.02
Replication of CLU, CR1, and PICALM associations with alzheimer disease. Arch Neurol (2010) 2.02
Lrrk2 and Lewy body disease. Ann Neurol (2006) 2.00
Translation initiator EIF4G1 mutations in familial Parkinson disease. Am J Hum Genet (2011) 2.00
Prominent phenotypic variability associated with mutations in Progranulin. Neurobiol Aging (2007) 2.00
Deletion of the ubiquitin ligase CHIP leads to the accumulation, but not the aggregation, of both endogenous phospho- and caspase-3-cleaved tau species. J Neurosci (2006) 1.98
Antemortem MRI based STructural Abnormality iNDex (STAND)-scores correlate with postmortem Braak neurofibrillary tangle stage. Neuroimage (2008) 1.98
Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS. Acta Neuropathol (2013) 1.95
Distinct anatomical subtypes of the behavioural variant of frontotemporal dementia: a cluster analysis study. Brain (2009) 1.93
Beta-amyloid burden is not associated with rates of brain atrophy. Ann Neurol (2008) 1.93
Voxel-based morphometry in autopsy proven PSP and CBD. Neurobiol Aging (2006) 1.92
Dimeric amyloid beta protein rapidly accumulates in lipid rafts followed by apolipoprotein E and phosphorylated tau accumulation in the Tg2576 mouse model of Alzheimer's disease. J Neurosci (2004) 1.92
TMEM106B protects C9ORF72 expansion carriers against frontotemporal dementia. Acta Neuropathol (2014) 1.90
Rates of cerebral atrophy differ in different degenerative pathologies. Brain (2007) 1.90
Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes. Acta Neuropathol (2009) 1.89
Antemortem diagnosis of frontotemporal lobar degeneration. Ann Neurol (2005) 1.85
TREM2 in neurodegeneration: evidence for association of the p.R47H variant with frontotemporal dementia and Parkinson's disease. Mol Neurodegener (2013) 1.83
Evaluation of alpha-synuclein immunohistochemical methods used by invited experts. Acta Neuropathol (2008) 1.81
Rapid eye movement sleep behavior disorder and subtypes in autopsy-confirmed dementia with Lewy bodies. Mov Disord (2011) 1.73
Incidental Lewy body disease and preclinical Parkinson disease. Arch Neurol (2008) 1.72
C9ORF72 repeat expansions in cases with previously identified pathogenic mutations. Neurology (2013) 1.70
In situ hybridization for detection of nocardial 16S rRNA: reactivity within intracellular inclusions in experimentally infected cynomolgus monkeys--and in Lewy body-containing human brain specimens. Exp Neurol (2003) 1.68
Argyrophilic grain disease is a sporadic 4-repeat tauopathy. J Neuropathol Exp Neurol (2002) 1.61
Ataxin-2 repeat-length variation and neurodegeneration. Hum Mol Genet (2011) 1.60
Neurofilament inclusion body disease: a new proteinopathy? Brain (2003) 1.59
Ultrastructural neuronal pathology in transgenic mice expressing mutant (P301L) human tau. J Neurocytol (2003) 1.58
SLC20A2 and THAP1 deletion in familial basal ganglia calcification with dystonia. Neurogenetics (2013) 1.58
Neuropathology underlying clinical variability in patients with synucleinopathies. Acta Neuropathol (2011) 1.57
Assembly of tau in transgenic animals expressing P301L tau: alteration of phosphorylation and solubility. J Neurochem (2002) 1.57
DNAJC13 mutations in Parkinson disease. Hum Mol Genet (2013) 1.54
Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress. Acta Neuropathol (2014) 1.54
Temporal lobar predominance of TDP-43 neuronal cytoplasmic inclusions in Alzheimer disease. Acta Neuropathol (2008) 1.53
An inhibitor of tau hyperphosphorylation prevents severe motor impairments in tau transgenic mice. Proc Natl Acad Sci U S A (2006) 1.52
Reduced C9orf72 gene expression in c9FTD/ALS is caused by histone trimethylation, an epigenetic event detectable in blood. Acta Neuropathol (2013) 1.52
Frontotemporal dementia and parkinsonism associated with the IVS1+1G->A mutation in progranulin: a clinicopathologic study. Brain (2006) 1.51
Frontotemporal lobar degeneration with ubiquitin-positive, but TDP-43-negative inclusions. Acta Neuropathol (2008) 1.49
Tau pathology in frontotemporal lobar degeneration with C9ORF72 hexanucleotide repeat expansion. Acta Neuropathol (2012) 1.48
Alpha-synuclein immunohistochemistry in two cases of co-occurring idiopathic Parkinson's disease and motor neuron disease. Mov Disord (2005) 1.48
Actin-binding proteins coronin-1a and IBA-1 are effective microglial markers for immunohistochemistry. J Histochem Cytochem (2007) 1.47
TDP-43 is a key player in the clinical features associated with Alzheimer's disease. Acta Neuropathol (2014) 1.47
Hereditary diffuse leukoencephalopathy with spheroids: clinical, pathologic and genetic studies of a new kindred. Acta Neuropathol (2006) 1.47
Evidence for a role of the rare p.A152T variant in MAPT in increasing the risk for FTD-spectrum and Alzheimer's diseases. Hum Mol Genet (2012) 1.46
Temporoparietal atrophy: a marker of AD pathology independent of clinical diagnosis. Neurobiol Aging (2009) 1.46
TDP-43 in aging and Alzheimer's disease - a review. Int J Clin Exp Pathol (2011) 1.46
Validation of the neuropathologic criteria of the third consortium for dementia with Lewy bodies for prospectively diagnosed cases. J Neuropathol Exp Neurol (2008) 1.45
Mimicking aspects of frontotemporal lobar degeneration and Lou Gehrig's disease in rats via TDP-43 overexpression. Mol Ther (2009) 1.42