Published in J Biol Chem on November 03, 2011
Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition). Autophagy (2016) 7.05
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron (2013) 3.63
Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43. Proc Natl Acad Sci U S A (2012) 1.69
Aggrephagy: selective disposal of protein aggregates by macroautophagy. Int J Cell Biol (2012) 1.52
CD47 deficiency confers cell and tissue radioprotection by activation of autophagy. Autophagy (2012) 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
TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets. Neurotherapeutics (2015) 1.15
RNA-mediated toxicity in neurodegenerative disease. Mol Cell Neurosci (2012) 1.07
Loss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin-2 to induce motor neuron dysfunction and cell death. EMBO J (2016) 0.99
Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice. Mol Neurobiol (2013) 0.98
Caenorhabditis elegans RNA-processing protein TDP-1 regulates protein homeostasis and life span. J Biol Chem (2012) 0.96
The Function of Autophagy in Neurodegenerative Diseases. Int J Mol Sci (2015) 0.95
Frontotemporal dementia: a bridge between dementia and neuromuscular disease. Ann N Y Acad Sci (2014) 0.93
Interplay of endoplasmic reticulum stress and autophagy in neurodegenerative disorders. Autophagy (2016) 0.88
Recent progress in the discovery of small molecules for the treatment of amyotrophic lateral sclerosis (ALS). Beilstein J Org Chem (2013) 0.87
Astrocytic TDP-43 pathology in Alexander disease. J Neurosci (2014) 0.85
Ubiquilin-2 (UBQLN2) binds with high affinity to the C-terminal region of TDP-43 and modulates TDP-43 levels in H4 cells: characterization of inhibition by nucleic acids and 4-aminoquinolines. Biochim Biophys Acta (2013) 0.84
Linking RNA Dysfunction and Neurodegeneration in Amyotrophic Lateral Sclerosis. Neurotherapeutics (2015) 0.83
Characterization of a series of 4-aminoquinolines that stimulate caspase-7 mediated cleavage of TDP-43 and inhibit its function. Biochimie (2012) 0.82
TDP-43 affects splicing profiles and isoform production of genes involved in the apoptotic and mitotic cellular pathways. Nucleic Acids Res (2015) 0.79
Mechanistic evaluation of a novel small molecule targeting mitochondria in pancreatic cancer cells. PLoS One (2013) 0.79
Proteasome dysfunction induces muscle growth defects and protein aggregation. J Cell Sci (2014) 0.79
RNA-processing protein TDP-43 regulates FOXO-dependent protein quality control in stress response. PLoS Genet (2014) 0.78
A systems biology investigation of neurodegenerative dementia reveals a pivotal role of autophagy. BMC Syst Biol (2014) 0.77
Activation of ER Stress and Autophagy Induced by TDP-43 A315T as Pathogenic Mechanism and the Corresponding Histological Changes in Skin as Potential Biomarker for ALS with the Mutation. Int J Biol Sci (2015) 0.77
Therapeutic and diagnostic challenges for frontotemporal dementia. Front Aging Neurosci (2014) 0.76
Tdp-43 cryptic exons are highly variable between cell types. Mol Neurodegener (2017) 0.76
Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia Continuum. Front Mol Neurosci (2017) 0.76
Energy Homeostasis and Abnormal RNA Metabolism in Amyotrophic Lateral Sclerosis. Front Cell Neurosci (2017) 0.75
Emerging connections between RNA and autophagy. Autophagy (2016) 0.75
Therapeutic effect of berberine on TDP-43-related pathogenesis in FTLD and ALS. J Biomed Sci (2016) 0.75
Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities? Front Mol Neurosci (2017) 0.75
Stress granules at the intersection of autophagy and ALS. Brain Res (2016) 0.75
TDP-43/HDAC6 axis promoted tumor progression and regulated nutrient deprivation-induced autophagy in glioblastoma. Oncotarget (2017) 0.75
Autophagy and Its Impact on Neurodegenerative Diseases: New Roles for TDP-43 and C9orf72. Front Mol Neurosci (2017) 0.75
TDP-43 expression influences amyloidβ plaque deposition and tau aggregation. Neurobiol Dis (2017) 0.75
LC3, a mammalian homologue of yeast Apg8p, is localized in autophagosome membranes after processing. EMBO J (2000) 41.48
Quantitative studies of the growth of mouse embryo cells in culture and their development into established lines. J Cell Biol (1963) 29.15
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science (2006) 27.96
Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice. Nature (2006) 24.39
p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy. J Biol Chem (2007) 21.73
Development by self-digestion: molecular mechanisms and biological functions of autophagy. Dev Cell (2004) 21.68
Loss of autophagy in the central nervous system causes neurodegeneration in mice. Nature (2006) 21.40
Impairment of starvation-induced and constitutive autophagy in Atg7-deficient mice. J Cell Biol (2005) 18.60
Autophagy in health and disease: a double-edged sword. Science (2004) 13.69
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
Protein degradation and protection against misfolded or damaged proteins. Nature (2003) 10.19
Regulation of mammalian autophagy in physiology and pathophysiology. Physiol Rev (2010) 6.35
AU-rich elements and associated factors: are there unifying principles? Nucleic Acids Res (2006) 6.22
Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43. Nat Neurosci (2011) 6.01
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. Hum Mol Genet (2010) 5.75
Characterizing the RNA targets and position-dependent splicing regulation by TDP-43. Nat Neurosci (2011) 5.24
Nuclear factor TDP-43 and SR proteins promote in vitro and in vivo CFTR exon 9 skipping. EMBO J (2001) 5.23
Cloning and characterization of a novel cellular protein, TDP-43, that binds to human immunodeficiency virus type 1 TAR DNA sequence motifs. J Virol (1995) 5.18
Autophagy inhibition compromises degradation of ubiquitin-proteasome pathway substrates. Mol Cell (2009) 5.18
Linking of autophagy to ubiquitin-proteasome system is important for the regulation of endoplasmic reticulum stress and cell viability. Am J Pathol (2007) 4.45
Isolation of microtubule protein from cultured mouse neuroblastoma cells. Proc Natl Acad Sci U S A (1970) 4.20
Structural diversity and functional implications of the eukaryotic TDP gene family. Genomics (2004) 3.51
Human, Drosophila, and C.elegans TDP43: nucleic acid binding properties and splicing regulatory function. J Mol Biol (2005) 3.33
Neuroblastoma x spinal cord (NSC) hybrid cell lines resemble developing motor neurons. Dev Dyn (1992) 3.14
TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor. J Neurochem (2007) 2.91
TDP-43 regulates its mRNA levels through a negative feedback loop. EMBO J (2010) 2.90
TAR DNA-binding protein 43 in neurodegenerative disease. Nat Rev Neurol (2010) 2.81
TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein. Mol Cell Neurosci (2007) 2.75
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
Mutations in TDP-43 link glycine-rich domain functions to amyotrophic lateral sclerosis. Hum Mol Genet (2009) 2.50
Depletion of TDP-43 affects Drosophila motoneurons terminal synapsis and locomotive behavior. FEBS Lett (2009) 2.48
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
TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis. Genesis (2010) 2.31
Ubiquilin functions in autophagy and is degraded by chaperone-mediated autophagy. Hum Mol Genet (2010) 2.25
Sequestosome 1/p62--more than just a scaffold. FEBS Lett (2006) 2.07
Higher order arrangement of the eukaryotic nuclear bodies. Proc Natl Acad Sci U S A (2002) 1.99
TDP-43 overexpression enhances exon 7 inclusion during the survival of motor neuron pre-mRNA splicing. J Biol Chem (2008) 1.98
Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis. Acta Neuropathol (2010) 1.96
Protein homeostasis and aging: The importance of exquisite quality control. Ageing Res Rev (2010) 1.84
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: an emerging new player in neurodegenerative diseases. Trends Mol Med (2008) 1.70
The multiple roles of TDP-43 in pre-mRNA processing and gene expression regulation. RNA Biol (2010) 1.66
Potentiation of amyotrophic lateral sclerosis (ALS)-associated TDP-43 aggregation by the proteasome-targeting factor, ubiquilin 1. J Biol Chem (2008) 1.58
Degradation of amyotrophic lateral sclerosis-linked mutant Cu,Zn-superoxide dismutase proteins by macroautophagy and the proteasome. J Biol Chem (2006) 1.57
Rapamycin rescues TDP-43 mislocalization and the associated low molecular mass neurofilament instability. J Biol Chem (2009) 1.53
Degradation of TDP-43 and its pathogenic form by autophagy and the ubiquitin-proteasome system. Neurosci Lett (2009) 1.53
The molecular links between TDP-43 dysfunction and neurodegeneration. Adv Genet (2009) 1.46
TDP-43 depletion induces neuronal cell damage through dysregulation of Rho family GTPases. J Biol Chem (2009) 1.44
Synergistic effect between proteasome and autophagosome in the clearance of polyubiquitinated TDP-43. J Neurosci Res (2010) 1.26
P62 and the sequestosome, a novel mechanism for protein metabolism. Arch Pharm Res (1998) 1.24
Ubiquitinated, p62 immunopositive cerebellar cortical neuronal inclusions are evident across the spectrum of TDP-43 proteinopathies but are only rarely additionally immunopositive for phosphorylation-dependent TDP-43. Neuropathology (2010) 1.13
Neuronal function and dysfunction of Drosophila dTDP. PLoS One (2011) 0.96
Structural diversity and functional implications of the eukaryotic TDP gene family. Genomics (2004) 3.51
TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor. J Neurochem (2007) 2.91
TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis. Genesis (2010) 2.31
Higher order arrangement of the eukaryotic nuclear bodies. Proc Natl Acad Sci U S A (2002) 1.99
TDP-43 overexpression enhances exon 7 inclusion during the survival of motor neuron pre-mRNA splicing. J Biol Chem (2008) 1.98
Rate of evolution in brain-expressed genes in humans and other primates. PLoS Biol (2007) 1.87
TDP-43: an emerging new player in neurodegenerative diseases. Trends Mol Med (2008) 1.70
Rapidly evolving genes in human. I. The glycophorins and their possible role in evading malaria parasites. Mol Biol Evol (2003) 1.35
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
Epigenetic regulation of the Drosophila chromosome 4 by the histone H3K9 methyltransferase dSETDB1. Proc Natl Acad Sci U S A (2007) 1.17
DNA methyltransferase gene dDnmt2 and longevity of Drosophila. J Biol Chem (2004) 1.10
The eukaryotic DNMT2 genes encode a new class of cytosine-5 DNA methyltransferases. J Biol Chem (2003) 1.09
Substitution rate and structural divergence of 5'UTR evolution: comparative analysis between human and cynomolgus monkey cDNAs. Mol Biol Evol (2005) 0.97
Neuronal function and dysfunction of Drosophila dTDP. PLoS One (2011) 0.96
Sumoylation of p45/NF-E2: nuclear positioning and transcriptional activation of the mammalian beta-like globin gene locus. Mol Cell Biol (2005) 0.93
Ankrd17, an ubiquitously expressed ankyrin factor, is essential for the vascular integrity during embryogenesis. FEBS Lett (2009) 0.89
Erythroid gene suppression by NF-kappa B. J Biol Chem (2003) 0.86
Developmental silencing of human zeta-globin gene expression is mediated by the transcriptional repressor RREB1. J Biol Chem (2010) 0.85
Analysis of the interaction between Zinc finger protein 179 (Znf179) and promyelocytic leukemia zinc finger (Plzf). J Biomed Sci (2013) 0.84
Actin-based modeling of a transcriptionally competent nuclear substructure induced by transcription inhibition. Exp Cell Res (2006) 0.82
Eukaryotic methyl-CpG-binding domain proteins and chromatin modification. Eukaryot Cell (2003) 0.81
PP2B-mediated dephosphorylation of c-Jun C terminus regulates phorbol ester-induced c-Jun/Sp1 interaction in A431 cells. Mol Biol Cell (2007) 0.81