Published in Int J Exp Pathol on February 01, 2013
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron (2013) 3.63
Mutant TDP-43 deregulates AMPK activation by PP2A in ALS models. PLoS One (2014) 1.41
Amyotrophic lateral sclerosis--a model of corticofugal axonal spread. Nat Rev Neurol (2013) 1.29
Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models. Hum Mol Genet (2013) 1.19
Dysregulated miRNA biogenesis downstream of cellular stress and ALS-causing mutations: a new mechanism for ALS. EMBO J (2015) 0.88
Prevention of intestinal obstruction reveals progressive neurodegeneration in mutant TDP-43 (A315T) mice. Mol Neurodegener (2014) 0.83
FTD and ALS--translating mouse studies into clinical trials. Nat Rev Neurol (2015) 0.82
From animal models to human disease: a genetic approach for personalized medicine in ALS. Acta Neuropathol Commun (2016) 0.80
The bowel and beyond: the enteric nervous system in neurological disorders. Nat Rev Gastroenterol Hepatol (2016) 0.80
Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS. Acta Neuropathol Commun (2015) 0.79
TDP-43/FUS in motor neuron disease: Complexity and challenges. Prog Neurobiol (2016) 0.79
A high-fat jelly diet restores bioenergetic balance and extends lifespan in the presence of motor dysfunction and lumbar spinal cord motor neuron loss in TDP-43A315T mutant C57BL6/J mice. Dis Model Mech (2016) 0.75
Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis. Acta Neuropathol (2017) 0.75
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Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science (2006) 27.96
Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science (2009) 13.45
Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science (2009) 12.99
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
TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis. Nat Genet (2008) 9.51
Molecular biology of amyotrophic lateral sclerosis: insights from genetics. Nat Rev Neurosci (2006) 6.53
TDP-43 A315T mutation in familial motor neuron disease. Ann Neurol (2008) 5.58
Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1. Science (1998) 5.23
Accelerated amyloid deposition in the brains of transgenic mice coexpressing mutant presenilin 1 and amyloid precursor proteins. Neuron (1997) 4.49
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci U S A (2009) 4.18
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
Motoneuron death triggered by a specific pathway downstream of Fas. potentiation by ALS-linked SOD1 mutations. Neuron (2002) 3.31
TDP-43 mutation in familial amyotrophic lateral sclerosis. Ann Neurol (2008) 2.92
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
Glutamate transporters in neurologic disease. Arch Neurol (2001) 1.80
Coincident thresholds of mutant protein for paralytic disease and protein aggregation caused by restrictively expressed superoxide dismutase cDNA. Neurobiol Dis (2005) 1.69
Functions of intermediate filaments in neuronal development and disease. J Neurobiol (2004) 1.63
Progranulin genetic variability contributes to amyotrophic lateral sclerosis. Neurology (2008) 1.56
Increased expression of the pro-inflammatory enzyme cyclooxygenase-2 in amyotrophic lateral sclerosis. Ann Neurol (2001) 1.55
Message and protein-level elevation of tumor necrosis factor alpha (TNF alpha) and TNF alpha-modulating cytokines in spinal cords of the G93A-SOD1 mouse model for amyotrophic lateral sclerosis. Neurobiol Dis (2003) 1.51
Progressive motor weakness in transgenic mice expressing human TDP-43. Neurobiol Dis (2010) 1.51
CHIP promotes proteasomal degradation of familial ALS-linked mutant SOD1 by ubiquitinating Hsp/Hsc70. J Neurochem (2004) 1.50
Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB-mediated pathogenic pathways. J Exp Med (2011) 1.46
Mimicking aspects of frontotemporal lobar degeneration and Lou Gehrig's disease in rats via TDP-43 overexpression. Mol Ther (2009) 1.42
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Thalidomide and lenalidomide extend survival in a transgenic mouse model of amyotrophic lateral sclerosis. J Neurosci (2006) 1.34
Focal dysfunction of the proteasome: a pathogenic factor in a mouse model of amyotrophic lateral sclerosis. J Neurochem (2004) 1.24
Protein aggregation in motor neurone disorders. Neuropathol Appl Neurobiol (2003) 1.14
Mitochondrial degeneration in amyotrophic lateral sclerosis. J Bioenerg Biomembr (2004) 1.02
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Relevance of oxidative injury in the pathogenesis of motor neuron diseases. Amyotroph Lateral Scler Other Motor Neuron Disord (2003) 1.01
Integrative role of cPLA with COX-2 and the effect of non-steriodal anti-inflammatory drugs in a transgenic mouse model of amyotrophic lateral sclerosis. J Neurochem (2005) 0.96
HO-1 induction in motor cortex and intestinal dysfunction in TDP-43 A315T transgenic mice. Brain Res (2012) 0.95
Rodent models of TDP-43 proteinopathy: investigating the mechanisms of TDP-43-mediated neurodegeneration. J Mol Neurosci (2011) 0.87
Programmed cell death in amyotrophic lateral sclerosis: a mechanism of pathogenic and therapeutic importance. Neurologist (2004) 0.86
Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice. J Biol Chem (2002) 2.68
Neural mitochondrial Ca2+ capacity impairment precedes the onset of motor symptoms in G93A Cu/Zn-superoxide dismutase mutant mice. J Neurochem (2006) 1.64
Impaired PGC-1alpha function in muscle in Huntington's disease. Hum Mol Genet (2009) 1.63
Additive neuroprotective effects of a histone deacetylase inhibitor and a catalytic antioxidant in a transgenic mouse model of amyotrophic lateral sclerosis. Neurobiol Dis (2005) 1.46
A new model for nonalcoholic steatohepatitis in the rat utilizing total enteral nutrition to overfeed a high-polyunsaturated fat diet. Am J Physiol Gastrointest Liver Physiol (2007) 1.39
Neuroprotective effects of the triterpenoid, CDDO methyl amide, a potent inducer of Nrf2-mediated transcription. PLoS One (2009) 1.29
Cell-permeable peptide antioxidants as a novel therapeutic approach in a mouse model of amyotrophic lateral sclerosis. J Neurochem (2006) 1.26
Additive neuroprotective effects of creatine and cyclooxygenase 2 inhibitors in a transgenic mouse model of amyotrophic lateral sclerosis. J Neurochem (2004) 1.17
N-acetylcysteine attenuates progression of liver pathology in a rat model of nonalcoholic steatohepatitis. J Nutr (2008) 1.15
Kallikrein 7 enhances pancreatic cancer cell invasion by shedding E-cadherin. Cancer (2007) 1.13
Neuroprotective effect of Nrf2/ARE activators, CDDO ethylamide and CDDO trifluoroethylamide, in a mouse model of amyotrophic lateral sclerosis. Free Radic Biol Med (2011) 1.13
Nitration of Hsp90 induces cell death. Proc Natl Acad Sci U S A (2013) 1.13
Stroke location and brain function in an embolic rabbit stroke model. J Vasc Interv Radiol (2010) 1.07
A novel systemically active caspase inhibitor attenuates the toxicities of MPTP, malonate, and 3NP in vivo. Neurobiol Dis (2004) 1.06
Effects of grape seed proanthocyanidin extract on oxidative stress induced by diabetes in rat kidney. Iran Biomed J (2011) 1.04
Oral glutamine protects against cyclophosphamide-induced cardiotoxicity in experimental rats through increase of cardiac glutathione. Nutrition (2009) 1.03
Nrf2/ARE Signaling Pathway: Key Mediator in Oxidative Stress and Potential Therapeutic Target in ALS. Neurol Res Int (2012) 1.02
Chondroitin sulfate glycosaminoglycans as major P-selectin ligands on metastatic breast cancer cell lines. Int J Cancer (2007) 1.02
Alginate oligosaccharide protects against endoplasmic reticulum- and mitochondrial-mediated apoptotic cell death and oxidative stress. Biomaterials (2011) 1.02
Lenalidomide (Revlimid) administration at symptom onset is neuroprotective in a mouse model of amyotrophic lateral sclerosis. Exp Neurol (2009) 0.99
Loss of Fas ligand-function improves survival in G93A-transgenic ALS mice. J Neurol Sci (2006) 0.99
Nrf2 and Nrf1 signaling and ER stress crosstalk: implication for proteasomal degradation and autophagy. Cell Mol Life Sci (2013) 0.96
Photothermal multispectral image cytometry for quantitative histology of nanoparticles and micrometastasis in intact, stained and selectively burned tissues. Cytometry A (2010) 0.95
Increased affinity for copper mediated by cysteine 111 in forms of mutant superoxide dismutase 1 linked to amyotrophic lateral sclerosis. Free Radic Biol Med (2007) 0.95
Desmoglein 2 is a substrate of kallikrein 7 in pancreatic cancer. BMC Cancer (2008) 0.94
Iron porphyrin treatment extends survival in a transgenic animal model of amyotrophic lateral sclerosis. J Neurochem (2003) 0.93
The matrix metalloproteinases inhibitor Ro 28-2653 [correction of Ro 26-2853] extends survival in transgenic ALS mice. Exp Neurol (2006) 0.93
Human recombinant vascular endothelial growth factor reduces necrosis and enhances hepatocyte regeneration in a mouse model of acetaminophen toxicity. J Pharmacol Exp Ther (2010) 0.92
Local host response to chlamydial urethral infection in male guinea pigs. Infect Immun (2010) 0.91
Essential role for neutrophils in pathogenesis and adaptive immunity in Chlamydia caviae ocular infections. Infect Immun (2011) 0.90
Acetaminophen-induced hepatotoxicity and protein nitration in neuronal nitric-oxide synthase knockout mice. J Pharmacol Exp Ther (2011) 0.90
Effects of long-term ethanol administration in a rat total enteral nutrition model of alcoholic liver disease. Am J Physiol Gastrointest Liver Physiol (2010) 0.90
Acetaminophen hepatotoxicity and HIF-1α induction in acetaminophen toxicity in mice occurs without hypoxia. Toxicol Appl Pharmacol (2011) 0.90
Conductive interstitial thermal therapy device for surgical margin ablation: in vivo verification of a theoretical model. Int J Hyperthermia (2007) 0.89
Reduction of spontaneous metastases through induction of carbohydrate cross-reactive apoptotic antibodies. J Immunol (2005) 0.89
The lipophilic metal chelators DP-109 and DP-460 are neuroprotective in a transgenic mouse model of amyotrophic lateral sclerosis. J Neurochem (2007) 0.88
Deficiency in surface expression of E-selectin ligand promotes lung colonization in a mouse model of breast cancer. Int J Cancer (2005) 0.86
Automatic identification of angiogenesis in double stained images of liver tissue. BMC Bioinformatics (2009) 0.86
Oral glutamine protects against acute doxorubicin-induced cardiotoxicity of tumor-bearing rats. J Nutr (2009) 0.86
Anticancer effect of calycopterin via PI3K/Akt and MAPK signaling pathways, ROS-mediated pathway and mitochondrial dysfunction in hepatoblastoma cancer (HepG2) cells. Mol Cell Biochem (2014) 0.85
Conductive interstitial thermal therapy (CITT) device evaluation in VX2 rabbit model. Technol Cancer Res Treat (2007) 0.85
Neurological assessment scores in rabbit embolic stroke models. Open Neurol J (2013) 0.84
Beta-amyloid 42 accumulation in the lumbar spinal cord motor neurons of amyotrophic lateral sclerosis patients. Neurobiol Dis (2005) 0.82
Interferon regulatory transcription factor 3 protects mice from uterine horn pathology during Chlamydia muridarum genital infection. Infect Immun (2011) 0.82
Interaction of 2-APB, dantrolene, and TDMT with IP3R and RyR modulates ER stress-induced programmed cell death I and II in neuron-like PC12 cells: an experimental and computational investigation. J Biomol Struct Dyn (2013) 0.81
Repression of multiple myeloma growth and preservation of bone with combined radiotherapy and anti-angiogenic agent. Radiat Res (2010) 0.80
Molecular dynamics guided design of tocoflexol: a new radioprotectant tocotrienol with enhanced bioavailability. Drug Dev Res (2013) 0.80
Immunomodulatory effects of radiofrequency ablation in a breast cancer model. Immunol Invest (2010) 0.79
An alternating focused ultrasound system for thermal therapy studies in small animals. Med Phys (2011) 0.79
Conductive interstitial thermal therapy (CITT) inhibits recurrence and metastasis in rabbit VX2 carcinoma model. Int J Hyperthermia (2009) 0.79
Feeding soy protein isolate and treatment with estradiol have different effects on mammary gland morphology and gene expression in weanling male and female rats. Physiol Genomics (2013) 0.78
Effect of trifluoperazine on toxicity, HIF-1α induction and hepatocyte regeneration in acetaminophen toxicity in mice. Toxicol Appl Pharmacol (2012) 0.77
Mammary gland morphology and gene expression signature of weanling male and female rats following exposure to exogenous estradiol. Exp Biol Med (Maywood) (2013) 0.76
Role of phosphodiesterase inhibitor Ibudilast in morphine-induced hippocampal injury. J Inj Violence Res (2013) 0.75
Amyotrophic lateral sclerosis and novel therapeutic strategies. Neurol Res Int (2012) 0.75
Mammary gland morphology and gene expression differ in female rats treated with 17β-estradiol or fed soy protein isolate. Endocrinology (2012) 0.75