Published in Mol Ther on May 21, 2013
Reversible behavioral phenotypes in a conditional mouse model of TDP-43 proteinopathies. J Neurosci (2014) 0.87
An insoluble frontotemporal lobar degeneration-associated TDP-43 C-terminal fragment causes neurodegeneration and hippocampus pathology in transgenic mice. Hum Mol Genet (2015) 0.86
Rodent Models of Amyotrophic Lateral Sclerosis. Curr Protoc Pharmacol (2015) 0.85
Preservation of forelimb function by UPF1 gene therapy in a rat model of TDP-43-induced motor paralysis. Gene Ther (2014) 0.84
AAV9 supports wide-scale transduction of the CNS and TDP-43 disease modeling in adult rats. Mol Ther Methods Clin Dev (2015) 0.81
Disease animal models of TDP-43 proteinopathy and their pre-clinical applications. Int J Mol Sci (2013) 0.77
Initial gene vector dosing for studying symptomatology of amyotrophic lateral sclerosis in non-human primates. J Med Primatol (2015) 0.76
The extreme N-terminus of TDP-43 mediates the cytoplasmic aggregation of TDP-43 and associated toxicity in vivo. Brain Res (2016) 0.76
Valproate Attenuates 25-kDa C-Terminal Fragment of TDP-43-Induced Neuronal Toxicity via Suppressing Endoplasmic Reticulum Stress and Activating Autophagy. Int J Biol Sci (2015) 0.76
Better Targeting, Better Efficiency for Wide-Scale Neuronal Transduction with the Synapsin Promoter and AAV-PHP.B. Front Mol Neurosci (2016) 0.75
Severe respiratory changes at end stage in a FUS-induced disease state in adult rats. BMC Neurosci (2016) 0.75
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
Clades of Adeno-associated viruses are widely disseminated in human tissues. J Virol (2004) 7.12
Intravascular AAV9 preferentially targets neonatal neurons and adult astrocytes. Nat Biotechnol (2008) 6.82
Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations. Ann Neurol (2007) 6.58
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
TDP-43 proteinopathy and motor neuron disease in chronic traumatic encephalopathy. J Neuropathol Exp Neurol (2010) 4.48
Self-complementary recombinant adeno-associated virus (scAAV) vectors promote efficient transduction independently of DNA synthesis. Gene Ther (2001) 4.35
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci U S A (2009) 4.18
Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity. Proc Natl Acad Sci U S A (2009) 3.65
Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation. J Biol Chem (2008) 3.56
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
Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis. J Neurosci (2010) 2.73
Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies. J Biol Chem (2009) 2.60
Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice. J Clin Invest (2011) 2.58
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
Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43. Hum Mol Genet (2009) 2.23
Phosphorylated TDP-43 in Alzheimer's disease and dementia with Lewy bodies. Acta Neuropathol (2009) 2.01
The pathophysiology of spasticity. Eur J Neurol (2002) 1.56
Rapamycin rescues TDP-43 mislocalization and the associated low molecular mass neurofilament instability. J Biol Chem (2009) 1.53
Dose and promoter effects of adeno-associated viral vector for green fluorescent protein expression in the rat brain. Exp Neurol (2002) 1.49
p62 accumulates and enhances aggregate formation in model systems of familial amyotrophic lateral sclerosis. J Biol Chem (2007) 1.48
Interaction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43. J Biol Chem (2010) 1.47
Mimicking aspects of frontotemporal lobar degeneration and Lou Gehrig's disease in rats via TDP-43 overexpression. Mol Ther (2009) 1.42
The advent of AAV9 expands applications for brain and spinal cord gene delivery. Expert Opin Biol Ther (2012) 1.30
Implications of the prion-related Q/N domains in TDP-43 and FUS. Prion (2011) 1.25
Expansive gene transfer in the rat CNS rapidly produces amyotrophic lateral sclerosis relevant sequelae when TDP-43 is overexpressed. Mol Ther (2010) 1.16
Both cytoplasmic and nuclear accumulations of the protein are neurotoxic in Drosophila models of TDP-43 proteinopathies. Neurobiol Dis (2010) 1.10
Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43. Brain (2012) 1.09
Inducible long-term gene expression in brain with adeno-associated virus gene transfer. Gene Ther (1998) 1.09
Cellular model of TAR DNA-binding protein 43 (TDP-43) aggregation based on its C-terminal Gln/Asn-rich region. J Biol Chem (2012) 1.08
Phosphorylation regulates proteasomal-mediated degradation and solubility of TAR DNA binding protein-43 C-terminal fragments. Mol Neurodegener (2010) 1.06
Phosphorylated and cleaved TDP-43 in ALS, FTLD and other neurodegenerative disorders and in cellular models of TDP-43 proteinopathy. Neuropathology (2010) 1.04
UBQLN2/P62 cellular recycling pathways in amyotrophic lateral sclerosis and frontotemporal dementia. Muscle Nerve (2012) 0.94
Cognitive decline typical of frontotemporal lobar degeneration in transgenic mice expressing the 25-kDa C-terminal fragment of TDP-43. Am J Pathol (2011) 0.92
Genetic strategies to study TDP-43 in rodents and to develop preclinical therapeutics for amyotrophic lateral sclerosis. Eur J Neurosci (2011) 0.88
Frontotemporal lobar degeneration-related proteins induce only subtle memory-related deficits when bilaterally overexpressed in the dorsal hippocampus. Exp Neurol (2011) 0.83
Antiretroviral preexposure prophylaxis for heterosexual HIV transmission in Botswana. N Engl J Med (2012) 20.03
US valuation of the EQ-5D health states: development and testing of the D1 valuation model. Med Care (2005) 9.89
Increased cancer-related mortality for patients with type 2 diabetes who use sulfonylureas or insulin. Diabetes Care (2006) 7.99
A meta-analysis of the association between adherence to drug therapy and mortality. BMJ (2006) 7.79
Prevention of rectal SHIV transmission in macaques by daily or intermittent prophylaxis with emtricitabine and tenofovir. PLoS Med (2008) 7.46
Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS. Neuron (2013) 5.91
Improved clinical outcomes associated with metformin in patients with diabetes and heart failure. Diabetes Care (2005) 4.78
Inhibitors of leucine-rich repeat kinase-2 protect against models of Parkinson's disease. Nat Med (2010) 4.45
Benefits and harms of antidiabetic agents in patients with diabetes and heart failure: systematic review. BMJ (2007) 3.95
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
Identification of the NF-E2-related factor-2-dependent genes conferring protection against oxidative stress in primary cortical astrocytes using oligonucleotide microarray analysis. J Biol Chem (2003) 3.61
Coordinate regulation of glutathione biosynthesis and release by Nrf2-expressing glia potently protects neurons from oxidative stress. J Neurosci (2003) 3.54
Progranulin mediates caspase-dependent cleavage of TAR DNA binding protein-43. J Neurosci (2007) 3.52
Self-reported health status of the general adult U.S. population as assessed by the EQ-5D and Health Utilities Index. Med Care (2005) 3.46
Physical activity level and health-related quality of life in the general adult population: a systematic review. Prev Med (2007) 3.45
The high-affinity HSP90-CHIP complex recognizes and selectively degrades phosphorylated tau client proteins. J Clin Invest (2007) 3.35
RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention. Neuron (2013) 3.25
Low-frequency HIV-1 drug resistance mutations and risk of NNRTI-based antiretroviral treatment failure: a systematic review and pooled analysis. JAMA (2011) 3.08
Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion. Sci Transl Med (2013) 2.89
Structural determinants of the cellular localization and shuttling of TDP-43. J Cell Sci (2008) 2.79
An important role of Nrf2-ARE pathway in the cellular defense mechanism. J Biochem Mol Biol (2004) 2.74
Pharmacodynamic characterization of chemopreventive triterpenoids as exceptionally potent inducers of Nrf2-regulated genes. Mol Cancer Ther (2007) 2.71
Prevalence of transmitted drug resistance associated mutations and HIV-1 subtypes in new HIV-1 diagnoses, U.S.-2006. AIDS (2010) 2.62
Nrf2, a multi-organ protector? FASEB J (2005) 2.61
Differential gene expression patterns revealed by oligonucleotide versus long cDNA arrays. Toxicol Sci (2002) 2.61
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
Evidence of suboptimal management of cardiovascular risk in patients with type 2 diabetes mellitus and symptomatic atherosclerosis. CMAJ (2004) 2.55
Nrf2-mediated neuroprotection in the MPTP mouse model of Parkinson's disease: Critical role for the astrocyte. Proc Natl Acad Sci U S A (2009) 2.54
Tar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue. PLoS One (2010) 2.49
A randomized trial of the effect of community pharmacist intervention on cholesterol risk management: the Study of Cardiovascular Risk Intervention by Pharmacists (SCRIP). Arch Intern Med (2002) 2.49
The Nrf2/ARE pathway as a potential therapeutic target in neurodegenerative disease. Antioxid Redox Signal (2009) 2.49
A controlled trial to increase detection and treatment of osteoporosis in older patients with a wrist fracture. Ann Intern Med (2004) 2.48
Nrf2 activation in astrocytes protects against neurodegeneration in mouse models of familial amyotrophic lateral sclerosis. J Neurosci (2008) 2.34
NF-E2-related factor-2 mediates neuroprotection against mitochondrial complex I inhibitors and increased concentrations of intracellular calcium in primary cortical neurons. J Biol Chem (2003) 2.33
Mortality after incident cancer in people with and without type 2 diabetes: impact of metformin on survival. Diabetes Care (2012) 2.32
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
Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons. Acta Neuropathol (2013) 2.26
Accelerated lipofuscinosis and ubiquitination in granulin knockout mice suggest a role for progranulin in successful aging. Am J Pathol (2010) 2.20
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
Impact of cancer on adherence to glucose-lowering drug treatment in individuals with diabetes. Diabetologia (2015) 1.96
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
TMEM106B protects C9ORF72 expansion carriers against frontotemporal dementia. Acta Neuropathol (2014) 1.90
A multicenter disease management program for hospitalized patients with heart failure. J Card Fail (2004) 1.90
Protection from mitochondrial complex II inhibition in vitro and in vivo by Nrf2-mediated transcription. Proc Natl Acad Sci U S A (2004) 1.89
Gene therapy with brain-derived neurotrophic factor as a protection: retinal ganglion cells in a rat glaucoma model. Invest Ophthalmol Vis Sci (2003) 1.83
Dose-response relation between sulfonylurea drugs and mortality in type 2 diabetes mellitus: a population-based cohort study. CMAJ (2006) 1.80
Multimorbidity prevalence and patterns across socioeconomic determinants: a cross-sectional survey. BMC Public Health (2012) 1.79
The impact of selecting a high hemoglobin target level on health-related quality of life for patients with chronic kidney disease: a systematic review and meta-analysis. Arch Intern Med (2009) 1.77
Health-related quality of life deficits associated with diabetes and comorbidities in a Canadian National Population Health Survey. Qual Life Res (2005) 1.76
HSP induction mediates selective clearance of tau phosphorylated at proline-directed Ser/Thr sites but not KXGS (MARK) sites. FASEB J (2006) 1.75
Enhancing primary care for complex patients. Demonstration project using multidisciplinary teams. Can Fam Physician (2004) 1.74
Developmental exposure to methylmercury alters learning and induces depression-like behavior in male mice. Toxicol Sci (2007) 1.72
C9ORF72 repeat expansions in cases with previously identified pathogenic mutations. Neurology (2013) 1.70
Agreement between patient and proxy assessments of health-related quality of life after stroke using the EQ-5D and Health Utilities Index. Stroke (2004) 1.66
Akt and CHIP coregulate tau degradation through coordinated interactions. Proc Natl Acad Sci U S A (2008) 1.66
CHIP regulates leucine-rich repeat kinase-2 ubiquitination, degradation, and toxicity. Proc Natl Acad Sci U S A (2009) 1.64
Long-term association between frailty and health-related quality of life among survivors of critical illness: a prospective multicenter cohort study. Crit Care Med (2015) 1.62
Responsiveness of generic health-related quality of life measures in stroke. Qual Life Res (2005) 1.62
Weight loss required by the severely obese to achieve clinically important differences in health-related quality of life: two-year prospective cohort study. BMC Med (2014) 1.61
How evidence-based are the recommendations in evidence-based guidelines? PLoS Med (2007) 1.60
Genetic dissection of systemic autoimmune disease in Nrf2-deficient mice. Physiol Genomics (2004) 1.60
Retrospective analysis of histopathologic prognostic factors after hepatectomy for intrahepatic cholangiocarcinoma. Cancer J (2009) 1.59
Type of antidepressant therapy and risk of type 2 diabetes in people with depression. Diabetes Res Clin Pract (2007) 1.59
The Nrf2-ARE cytoprotective pathway in astrocytes. Expert Rev Mol Med (2009) 1.57
Neurotoxic effects of TDP-43 overexpression in C. elegans. Hum Mol Genet (2010) 1.56
The role of tau in neurodegeneration. Mol Neurodegener (2009) 1.54
Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress. Acta Neuropathol (2014) 1.54
Reduced C9orf72 gene expression in c9FTD/ALS is caused by histone trimethylation, an epigenetic event detectable in blood. Acta Neuropathol (2013) 1.52
Type 2 diabetes does not increase risk of depression. CMAJ (2006) 1.51
Lack of neurodegeneration in transgenic mice overexpressing mutant amyloid precursor protein is associated with increased levels of transthyretin and the activation of cell survival pathways. J Neurosci (2002) 1.51
Use of thiazolidinediones and the risk of bladder cancer among people with type 2 diabetes: a meta-analysis. CMAJ (2012) 1.50
Health literacy and health outcomes in diabetes: a systematic review. J Gen Intern Med (2012) 1.49
Radiologic and patient-reported functional outcomes in an elderly cohort with conservatively treated distal radius fractures. J Hand Surg Am (2004) 1.48
The cost of major comorbidity in people with diabetes mellitus. CMAJ (2003) 1.47
Compliance with clinical practice guidelines for type 2 diabetes in rural patients: treatment gaps and opportunities for improvement. Pharmacotherapy (2003) 1.47
TDP-43 is a key player in the clinical features associated with Alzheimer's disease. Acta Neuropathol (2014) 1.47
Pioglitazone and bladder cancer risk: a multipopulation pooled, cumulative exposure analysis. Diabetologia (2014) 1.46
Nrf transcription factors in keratinocytes are essential for skin tumor prevention but not for wound healing. Mol Cell Biol (2006) 1.45
Genetic alterations in colorectal cancer. Gastrointest Cancer Res (2012) 1.44
Identification of potential protein interactors of Lrrk2. Parkinsonism Relat Disord (2007) 1.44
Atenolol as initial antihypertensive therapy: an observational study comparing first-line agents. J Hypertens (2007) 1.44
A genomic screen for activators of the antioxidant response element. Proc Natl Acad Sci U S A (2007) 1.44
The effectiveness of beta-blockers after myocardial infarction in patients with type 2 diabetes. Diabetes Care (2005) 1.43
Nrf2-mediated protection against 6-hydroxydopamine. Brain Res (2007) 1.42
Mimicking aspects of frontotemporal lobar degeneration and Lou Gehrig's disease in rats via TDP-43 overexpression. Mol Ther (2009) 1.42
Primary amebic meningoencephalitis deaths associated with sinus irrigation using contaminated tap water. Clin Infect Dis (2012) 1.41
Phosphorylation of tau antagonizes apoptosis by stabilizing beta-catenin, a mechanism involved in Alzheimer's neurodegeneration. Proc Natl Acad Sci U S A (2007) 1.41
Homozygosity for the C9orf72 GGGGCC repeat expansion in frontotemporal dementia. Acta Neuropathol (2013) 1.41
Mesenchymal stem cells as a vehicle for targeted delivery of CRAds to lung metastases of breast carcinoma. Breast Cancer Res Treat (2007) 1.41