TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease

Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

2006 • 5,928 citations

Frontotemporal lobar degeneration

1998 • 4,937 citations

TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

2006 • 2,449 citations

Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17

2006 • 1,929 citations

hnRNP PROTEINS AND THE BIOGENESIS OF mRNA

1993 • 1,486 citations

A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy

1999 • 1,418 citations

The prevalence of frontotemporal dementia

2002 • 1,117 citations

Molecular biology of amyotrophic lateral sclerosis: insights from genetics

2006 • 1,071 citations

Correlation between severity and SMN protein level in spinal muscular atrophy

1997 • 1,052 citations

Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia

2005 • 783 citations

Cloning and characterization of a novel cellular protein, TDP-43, that binds to human immunodeficiency virus type 1 TAR DNA sequence motifs

1995 • 713 citations

The overlap of amyotrophic lateral sclerosis and frontotemporal dementia

2002 • 664 citations

The prevalence and causes of dementia in people under the age of 65 years

2003 • 615 citations

Nuclear factor TDP-43 and SR proteins promote in vitro and in vivo CFTR exon 9 skipping

2001 • 605 citations

Clinicopathological correlates in frontotemporal dementia

2004 • 578 citations

TDP-43 in Familial and Sporadic Frontotemporal Lobar Degeneration with Ubiquitin Inclusions

2007 • 488 citations

Frontotemporal dementia: Clinicopathological correlations

2006 • 468 citations

TDP-43 Binds Heterogeneous Nuclear Ribonucleoprotein A/B through Its C-terminal Tail

2005 • 440 citations

Frontotemporal Lobar Degeneration

2005 • 404 citations

NEURODEGENERATIVE DISEASES: New Concepts of Pathogenesis and Their Therapeutic Implications

2006 • 401 citations

Familial amyotrophic lateral sclerosis with frontotemporal dementia is linked to a locus on chromosome 9p13.2–21.3

2006 • 388 citations

A locus on chromosome 9p confers susceptibility to ALS and frontotemporal dementia

2006 • 348 citations

Human, Drosophila, and C.elegans TDP43: Nucleic Acid Binding Properties and Splicing Regulatory Function

2005 • 344 citations

UBIQUITIN-IMMUNOREACTIVE INTRANEURONAL INCLUSIONS IN AMYOTROPHIC LATERAL SCLEROSIS

1991 • 321 citations

Heterogeneity of ubiquitin pathology in frontotemporal lobar degeneration: classification and relation to clinical phenotype

2006 • 317 citations

TDP-43 in the Ubiquitin Pathology of Frontotemporal Dementia With VCP Gene Mutations

2007 • 316 citations

Pathological Heterogeneity of Frontotemporal Lobar Degeneration with Ubiquitin-Positive Inclusions Delineated by Ubiquitin Immunohistochemistry and Novel Monoclonal Antibodies

2006 • 313 citations

Structural diversity and functional implications of the eukaryotic TDP gene family

2003 • 293 citations

The neuropathology of frontotemporal lobar degeneration caused by mutations in the progranulin gene

2006 • 291 citations

Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43

2007 • 290 citations

TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation

2007 • 288 citations

Genetics of familial and sporadic amyotrophic lateral sclerosis

2006 • 244 citations

TDP-43-Positive White Matter Pathology in Frontotemporal Lobar Degeneration With Ubiquitin-Positive Inclusions

2007 • 221 citations

Depletion of TDP 43 overrides the need for exonic and intronic splicing enhancers in the human apoA-II gene

2005 • 218 citations

Novel Ubiquitin Neuropathology in Frontotemporal Dementia WithValosin-Containing ProteinGene Mutations

2006 • 213 citations

Progranulin in frontotemporal lobar degeneration and neuroinflammation

2007 • 212 citations

The Role of Hypothermia in the Management of Severe Brain Injury

2002 • 196 citations

Frontotemporal lobar degeneration with motor neuron disease-type inclusions predominates in 76 cases of frontotemporal degeneration

2004 • 186 citations

Neuropathologic Features of Frontotemporal Lobar Degeneration With Ubiquitin-Positive Inclusions With Progranulin Gene (PGRN) Mutations

2007 • 185 citations

Higher order arrangement of the eukaryotic nuclear bodies

2002 • 180 citations

Mutant valosin‐containing protein causes a novel type of frontotemporal dementia

2005 • 175 citations

Familial frontotemporal dementia with ubiquitin-positive inclusions is linked to chromosome 17q21-22

2001 • 160 citations

Histopathological changes underlying frontotemporal lobar degeneration with clinicopathological correlation

2005 • 137 citations

Familial frontotemporal dementia with ubiquitin-positive, tau-negative inclusions

2000 • 135 citations

Tau negative frontal lobe dementia at 17q21: significant finemapping of the candidate region to a 4.8 cM interval

2002 • 107 citations

Recent advances in the genetics of amyotrophic lateral sclerosis and frontotemporal dementia: common pathways in neurodegenerative disease

2006 • 85 citations

TDP43 depletion rescues aberrant CFTR exon 9 skipping

2006 • 82 citations

cis-Requirement for the maintenance of round spermatid-specific transcription

2006 • 76 citations

Analysis of IFT74as a candidate gene for chromosome 9p-linked ALS-FTD

2006 • 73 citations

Progranulin Mutations in Ubiquitin-Positive Frontotemporal Dementia Linked to Chromosome 17q21

2006 • 64 citations

Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degeneration

2007 • 58 citations

Frontotemporal dementia

2002 • 44 citations

Tau negative frontal lobe dementia at 17q21: Significant finemapping of the candidate region to a 4.8 cm interval

2002 • 17 citations

Construction of a Detailed Physical and Transcript Map of the FTDP-17 Candidate Region on Chromosome 17q21

1999 • 9 citations