Published in J Biol Chem on August 14, 2008
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. Hum Mol Genet (2010) 5.75
Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration. Nat Rev Neurosci (2011) 2.84
Identification of neuronal RNA targets of TDP-43-containing ribonucleoprotein complexes. J Biol Chem (2010) 2.72
ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS. Proc Natl Acad Sci U S A (2010) 2.65
Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies. J Biol Chem (2009) 2.60
TDP-43 is a developmentally regulated protein essential for early embryonic development. J Biol Chem (2009) 2.40
Knockdown of transactive response DNA-binding protein (TDP-43) downregulates histone deacetylase 6. EMBO J (2009) 2.37
Clinico-pathological features in amyotrophic lateral sclerosis with expansions in C9ORF72. Brain (2012) 2.28
Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism. Proc Natl Acad Sci U S A (2010) 2.05
Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis. Acta Neuropathol (2010) 1.96
Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice. Proc Natl Acad Sci U S A (2010) 1.92
Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseases. J Neurol (2009) 1.85
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene. PLoS Genet (2010) 1.81
Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U. J Exp Med (2010) 1.78
Functional mapping of the interaction between TDP-43 and hnRNP A2 in vivo. Nucleic Acids Res (2009) 1.77
TDP-43 is directed to stress granules by sorbitol, a novel physiological osmotic and oxidative stressor. Mol Cell Biol (2010) 1.64
TARDBP 3'-UTR variant in autopsy-confirmed frontotemporal lobar degeneration with TDP-43 proteinopathy. Acta Neuropathol (2009) 1.56
Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: a spectrum of TDP-43 proteinopathies. Neuropathology (2010) 1.53
Characterization of alternative isoforms and inclusion body of the TAR DNA-binding protein-43. J Biol Chem (2009) 1.44
TDP-43 depletion induces neuronal cell damage through dysregulation of Rho family GTPases. J Biol Chem (2009) 1.44
TDP-43 functions and pathogenic mechanisms implicated in TDP-43 proteinopathies. Trends Mol Med (2011) 1.38
The splicing regulator Sam68 binds to a novel exonic splicing silencer and functions in SMN2 alternative splicing in spinal muscular atrophy. EMBO J (2010) 1.28
Molecular basis of UG-rich RNA recognition by the human splicing factor TDP-43. Nat Struct Mol Biol (2013) 1.25
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TDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signaling. Hum Mol Genet (2011) 1.19
TDP-43-based animal models of neurodegeneration: new insights into ALS pathology and pathophysiology. Neurodegener Dis (2010) 1.19
TDP-43, an ALS linked protein, regulates fat deposition and glucose homeostasis. PLoS One (2013) 1.14
Regulation of autophagy by neuropathological protein TDP-43. J Biol Chem (2011) 1.10
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RNA processing pathways in amyotrophic lateral sclerosis. Neurogenetics (2010) 1.08
Redox signalling directly regulates TDP-43 via cysteine oxidation and disulphide cross-linking. EMBO J (2011) 1.07
The truncated C-terminal RNA recognition motif of TDP-43 protein plays a key role in forming proteinaceous aggregates. J Biol Chem (2013) 1.05
Sustained expression of TDP-43 and FUS in motor neurons in rodent's lifetime. Int J Biol Sci (2010) 1.01
TDP-43 regulates global translational yield by splicing of exon junction complex component SKAR. Nucleic Acids Res (2011) 1.01
Hyperphosphorylation as a defense mechanism to reduce TDP-43 aggregation. PLoS One (2011) 1.00
Neuronal function and dysfunction of Drosophila dTDP. PLoS One (2011) 0.96
Caenorhabditis elegans RNA-processing protein TDP-1 regulates protein homeostasis and life span. J Biol Chem (2012) 0.96
The testis-specific double bromodomain-containing protein BRDT forms a complex with multiple spliceosome components and is required for mRNA splicing and 3'-UTR truncation in round spermatids. Nucleic Acids Res (2012) 0.94
Alternative splicing in spinal muscular atrophy underscores the role of an intron definition model. RNA Biol (2011) 0.93
SMN-inducing compounds for the treatment of spinal muscular atrophy. Future Med Chem (2012) 0.90
RNA-binding proteins in neurodegenerative disease: TDP-43 and beyond. Wiley Interdiscip Rev RNA (2011) 0.90
Neurodegeneration-associated TDP-43 interacts with fragile X mental retardation protein (FMRP)/Staufen (STAU1) and regulates SIRT1 expression in neuronal cells. J Biol Chem (2012) 0.89
Drosophila TDP-43 dysfunction in glia and muscle cells cause cytological and behavioural phenotypes that characterize ALS and FTLD. Hum Mol Genet (2013) 0.89
TDP-43 potentiates alpha-synuclein toxicity to dopaminergic neurons in transgenic mice. Int J Biol Sci (2011) 0.88
Rodent models of TDP-43 proteinopathy: investigating the mechanisms of TDP-43-mediated neurodegeneration. J Mol Neurosci (2011) 0.87
TDP-43 knockdown impairs neurite outgrowth dependent on its target histone deacetylase 6. Mol Neurodegener (2011) 0.87
Brain pathology in myotonic dystrophy: when tauopathy meets spliceopathy and RNAopathy. Front Mol Neurosci (2014) 0.87
Alteration of POLDIP3 splicing associated with loss of function of TDP-43 in tissues affected with ALS. PLoS One (2012) 0.86
Comparison of parallel high-throughput RNA sequencing between knockout of TDP-43 and its overexpression reveals primarily nonreciprocal and nonoverlapping gene expression changes in the central nervous system of Drosophila. G3 (Bethesda) (2012) 0.86
TDP-43 loss of function increases TFEB activity and blocks autophagosome-lysosome fusion. EMBO J (2015) 0.86
Transgenic animal models of neurodegeneration based on human genetic studies. J Neural Transm (Vienna) (2010) 0.85
Targeting SR proteins improves SMN expression in spinal muscular atrophy cells. PLoS One (2014) 0.83
Low molecular weight species of TDP-43 generated by abnormal splicing form inclusions in amyotrophic lateral sclerosis and result in motor neuron death. Acta Neuropathol (2015) 0.83
Protein methylation and stress granules: posttranslational remodeler or innocent bystander? Mol Biol Int (2011) 0.83
Presynaptic localization of Smn and hnRNP R in axon terminals of embryonic and postnatal mouse motoneurons. PLoS One (2014) 0.81
SAM68 is a physiological regulator of SMN2 splicing in spinal muscular atrophy. J Cell Biol (2015) 0.80
Mass spectrometric analysis of accumulated TDP-43 in amyotrophic lateral sclerosis brains. Sci Rep (2016) 0.80
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The neuroprotective drug riluzole acts via small conductance Ca2+-activated K+ channels to ameliorate defects in spinal muscular atrophy models. J Neurosci (2013) 0.79
Splicing regulation in spinal muscular atrophy by an RNA structure formed by long-distance interactions. Ann N Y Acad Sci (2015) 0.79
RNA-processing protein TDP-43 regulates FOXO-dependent protein quality control in stress response. PLoS Genet (2014) 0.78
TDP-43 identified from a genome wide RNAi screen for SOD1 regulators. PLoS One (2012) 0.78
Similar dose-dependence of motor neuron cell death caused by wild type human TDP-43 and mutants with ALS-associated amino acid substitutions. J Biomed Sci (2013) 0.78
Co-regulation of mRNA translation by TDP-43 and Fragile X Syndrome protein FMRP. Acta Neuropathol (2016) 0.76
Cytoplasmic translocation, aggregation, and cleavage of TDP-43 by enteroviral proteases modulate viral pathogenesis. Cell Death Differ (2015) 0.76
Altered RNA metabolism and amyotrophic lateral sclerosis. Ann Indian Acad Neurol (2011) 0.76
Transcriptomic Changes Due to Cytoplasmic TDP-43 Expression Reveal Dysregulation of Histone Transcripts and Nuclear Chromatin. PLoS One (2015) 0.76
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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
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Disruption of an SF2/ASF-dependent exonic splicing enhancer in SMN2 causes spinal muscular atrophy in the absence of SMN1. Nat Genet (2002) 5.80
The essential pre-mRNA splicing factor SF2 influences 5' splice site selection by activating proximal sites. Cell (1990) 5.67
Nuclear factor TDP-43 and SR proteins promote in vitro and in vivo CFTR exon 9 skipping. EMBO J (2001) 5.23
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A single nucleotide difference that alters splicing patterns distinguishes the SMA gene SMN1 from the copy gene SMN2. Hum Mol Genet (1999) 5.09
Characterization and functional implications of the RNA binding properties of nuclear factor TDP-43, a novel splicing regulator of CFTR exon 9. J Biol Chem (2001) 4.87
TDP-43 binds heterogeneous nuclear ribonucleoprotein A/B through its C-terminal tail: an important region for the inhibition of cystic fibrosis transmembrane conductance regulator exon 9 splicing. J Biol Chem (2005) 3.99
A negative element in SMN2 exon 7 inhibits splicing in spinal muscular atrophy. Nat Genet (2003) 3.84
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SMN oligomerization defect correlates with spinal muscular atrophy severity. Nat Genet (1998) 3.51
An exonic enhancer is required for inclusion of an essential exon in the SMA-determining gene SMN. Hum Mol Genet (2000) 3.41
TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor. J Neurochem (2007) 2.91
Htra2-beta 1 stimulates an exonic splicing enhancer and can restore full-length SMN expression to survival motor neuron 2 (SMN2). Proc Natl Acad Sci U S A (2000) 2.72
Depletion of TDP 43 overrides the need for exonic and intronic splicing enhancers in the human apoA-II gene. Nucleic Acids Res (2005) 2.55
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hnRNP-G promotes exon 7 inclusion of survival motor neuron (SMN) via direct interaction with Htra2-beta1. Hum Mol Genet (2002) 1.84
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hnRNP A1 functions with specificity in repression of SMN2 exon 7 splicing. Hum Mol Genet (2007) 1.69
An extended inhibitory context causes skipping of exon 7 of SMN2 in spinal muscular atrophy. Biochem Biophys Res Commun (2004) 1.56
TDP-43 in differential diagnosis of motor neuron disorders. Acta Neuropathol (2007) 1.56
SRp30c-dependent stimulation of survival motor neuron (SMN) exon 7 inclusion is facilitated by a direct interaction with hTra2 beta 1. Hum Mol Genet (2002) 1.53
TDP43 depletion rescues aberrant CFTR exon 9 skipping. FEBS Lett (2006) 1.45
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Tau exons 2 and 10, which are misregulated in neurodegenerative diseases, are partly regulated by silencers which bind a SRp30c.SRp55 complex that either recruits or antagonizes htra2beta1. J Biol Chem (2005) 1.15
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Enhancer-dependent splicing of FGFR1 alpha-exon is repressed by RNA interference-mediated down-regulation of SRp55. Cancer Res (2004) 0.89
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TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis. Genesis (2010) 2.31
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The DEAD-box RNA helicase DDX3 associates with export messenger ribonucleoproteins as well as tip-associated protein and participates in translational control. Mol Biol Cell (2008) 1.67
The pioneer round of translation: features and functions. Cell (2010) 1.66
Hypophosphorylated ASF/SF2 binds TAP and is present in messenger ribonucleoproteins. J Biol Chem (2004) 1.53
A novel splicing regulator shares a nuclear import pathway with SR proteins. EMBO J (2003) 1.51
Cell stress modulates the function of splicing regulatory protein RBM4 in translation control. Proc Natl Acad Sci U S A (2007) 1.48
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The WW domain-containing proteins interact with the early spliceosome and participate in pre-mRNA splicing in vivo. Mol Cell Biol (2004) 1.33
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The RNA binding protein hnRNP Q modulates the utilization of exon 7 in the survival motor neuron 2 (SMN2) gene. Mol Cell Biol (2008) 1.27
Targeted depletion of TDP-43 expression in the spinal cord motor neurons leads to the development of amyotrophic lateral sclerosis-like phenotypes in mice. J Biol Chem (2012) 1.26
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Nuclear Pnn/DRS protein binds to spliced mRNPs and participates in mRNA processing and export via interaction with RNPS1. Mol Cell Biol (2003) 1.19
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Regulation of autophagy by neuropathological protein TDP-43. J Biol Chem (2011) 1.10
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RBM4 down-regulates PTB and antagonizes its activity in muscle cell-specific alternative splicing. J Cell Biol (2011) 1.04
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