Published in Ann Neurol on July 01, 2003
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Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy. Nat Med (2003) 1.73
Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan. Amyotroph Lateral Scler (2006) 1.73
The association of methamphetamine use and cardiomyopathy in young patients. Am J Med (2007) 1.70
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Spatiotemporal recapitulation of central nervous system development by murine embryonic stem cell-derived neural stem/progenitor cells. Stem Cells (2008) 1.67
Differential expression of inflammation- and apoptosis-related genes in spinal cords of a mutant SOD1 transgenic mouse model of familial amyotrophic lateral sclerosis. J Neurochem (2002) 1.63
Gene expression profile of spinal motor neurons in sporadic amyotrophic lateral sclerosis. Ann Neurol (2005) 1.59
Position-dependent FUS-RNA interactions regulate alternative splicing events and transcriptions. Sci Rep (2012) 1.58
Clinical and neuropathological correlates of Lewy body disease. Acta Neuropathol (2003) 1.58
Natural history of spinal and bulbar muscular atrophy (SBMA): a study of 223 Japanese patients. Brain (2006) 1.57
Disulfide bond mediates aggregation, toxicity, and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD1. J Biol Chem (2007) 1.57
Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein. J Neurosci (2003) 1.54
Molecular hydrogen is protective against 6-hydroxydopamine-induced nigrostriatal degeneration in a rat model of Parkinson's disease. Neurosci Lett (2009) 1.54
Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Hum Mol Genet (2004) 1.52
Suppression of UV-induced wrinkle formation by induction of HSP70 expression in mice. J Invest Dermatol (2012) 1.46
TDP-43 depletion induces neuronal cell damage through dysregulation of Rho family GTPases. J Biol Chem (2009) 1.44
Phase 2 trial of leuprorelin in patients with spinal and bulbar muscular atrophy. Ann Neurol (2009) 1.43
Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease. Proc Natl Acad Sci U S A (2005) 1.42
Viral delivery of miR-196a ameliorates the SBMA phenotype via the silencing of CELF2. Nat Med (2012) 1.41
Early pathologic changes in hereditary diffuse leukoencephalopathy with spheroids. J Neuropathol Exp Neurol (2014) 1.38
Clinical effects of tumor-associated macrophages and dendritic cells on renal cell carcinoma. Anticancer Res (2003) 1.37
Genetic evidence for a protective role for heat shock factor 1 and heat shock protein 70 against colitis. J Biol Chem (2007) 1.36
CHIP overexpression reduces mutant androgen receptor protein and ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model. J Neurosci (2007) 1.34
Widespread nuclear and cytoplasmic accumulation of mutant androgen receptor in SBMA patients. Brain (2005) 1.33
Stoichiometric interactions between cyanobacterial clock proteins KaiA and KaiC. Biochem Biophys Res Commun (2004) 1.32
Inverse relation between osteoporosis and spondylosis in postmenopausal women as evaluated by bone mineral density and semiquantitative scoring of spinal degeneration. Spine (Phila Pa 1976) (2003) 1.26
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17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse. Hum Mol Genet (2008) 1.25
Reversible disruption of dynactin 1-mediated retrograde axonal transport in polyglutamine-induced motor neuron degeneration. J Neurosci (2006) 1.25
Early ureteral catheter removal after ureteroscopic lithotripsy using ureteral access sheath. Urolithiasis (2012) 1.25
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Spliceosome integrity is defective in the motor neuron diseases ALS and SMA. EMBO Mol Med (2013) 1.22
Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity. J Biol Chem (2002) 1.19
Type I (transthyretin Met30) familial amyloid polyneuropathy in Japan: early- vs late-onset form. Arch Neurol (2002) 1.19
Pathogenesis, animal models and therapeutics in spinal and bulbar muscular atrophy (SBMA). Exp Neurol (2006) 1.18
Modulation of Hsp90 function in neurodegenerative disorders: a molecular-targeted therapy against disease-causing protein. J Mol Med (Berl) (2006) 1.16
Cerebellar ataxia rehabilitation trial in degenerative cerebellar diseases. Neurorehabil Neural Repair (2011) 1.14
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Suppression of Alzheimer's disease-related phenotypes by expression of heat shock protein 70 in mice. J Neurosci (2011) 1.14
Dietary restriction ameliorates diabetic nephropathy through anti-inflammatory effects and regulation of the autophagy via restoration of Sirt1 in diabetic Wistar fatty (fa/fa) rats: a model of type 2 diabetes. Exp Diabetes Res (2011) 1.14
Metallurgical characterization of a new nickel-titanium wire for rotary endodontic instruments. J Endod (2009) 1.12
Peripheral Nerve Society Guideline on the classification, diagnosis, investigation, and immunosuppressive therapy of non-systemic vasculitic neuropathy: executive summary. J Peripher Nerv Syst (2010) 1.11
Pathogenesis and therapy of spinal and bulbar muscular atrophy (SBMA). Prog Neurobiol (2012) 1.10
Prevention of UVB radiation-induced epidermal damage by expression of heat shock protein 70. J Biol Chem (2009) 1.09
A role for HSP70 in protecting against indomethacin-induced gastric lesions. J Biol Chem (2009) 1.09
Disrupted transforming growth factor-beta signaling in spinal and bulbar muscular atrophy. J Neurosci (2010) 1.09
Blockade of gap junction hemichannel suppresses disease progression in mouse models of amyotrophic lateral sclerosis and Alzheimer's disease. PLoS One (2011) 1.08
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c-Ret-mediated hearing loss in mice with Hirschsprung disease. Proc Natl Acad Sci U S A (2010) 1.07
The potential of GPNMB as novel neuroprotective factor in amyotrophic lateral sclerosis. Sci Rep (2012) 1.06
Rapidly developing weakness mimicking Guillain-Barré syndrome in beriberi neuropathy: two case reports. Nutrition (2008) 1.06
Clinicopathological features of neuropathy associated with lymphoma. Brain (2013) 1.06
Ureteroscopy-assisted retrograde nephrostomy (UARN) for an incomplete double ureter. Urol Res (2012) 1.06
Lipid peroxidation and advanced glycation end products in the brain in normal aging and in Alzheimer's disease. Acta Neuropathol (2002) 1.05
Analysis of C9orf72 repeat expansion in 563 Japanese patients with amyotrophic lateral sclerosis. Neurobiol Aging (2012) 1.05
Loss of TDP-43 causes age-dependent progressive motor neuron degeneration. Brain (2013) 1.05
Pilot study of H₂ therapy in Parkinson's disease: a randomized double-blind placebo-controlled trial. Mov Disord (2013) 1.04
Hsp70 and Hsp40 improve neurite outgrowth and suppress intracytoplasmic aggregate formation in cultured neuronal cells expressing mutant SOD1. Brain Res (2002) 1.04
Oncogenic activation of c-Myb correlates with a loss of negative regulation by TIF1beta and Ski. J Biol Chem (2004) 1.04
CAG repeat size correlates to electrophysiological motor and sensory phenotypes in SBMA. Brain (2007) 1.04
Correlation between pyramidal tract degeneration and widespread white matter involvement in amyotrophic lateral sclerosis: a study with tractography and diffusion-tensor imaging. Amyotroph Lateral Scler (2009) 1.03
Structural analysis of O-glycopeptides employing negative- and positive-ion multi-stage mass spectra obtained by collision-induced and electron-capture dissociations in linear ion trap time-of-flight mass spectrometry. Rapid Commun Mass Spectrom (2007) 1.03
Mitochondrial localization of mutant superoxide dismutase 1 triggers caspase-dependent cell death in a cellular model of familial amyotrophic lateral sclerosis. J Biol Chem (2002) 1.03
Macroautophagy is regulated by the UPR-mediator CHOP and accentuates the phenotype of SBMA mice. PLoS Genet (2011) 1.02
A low-grade extraskeletal osteosarcoma. Skeletal Radiol (2002) 1.02
Mortality and morbidity in peripheral neuropathy associated Churg-Strauss syndrome and microscopic polyangiitis. J Rheumatol (2002) 1.02
Microarray analysis of gene expression by skeletal muscle of three mouse models of Kennedy disease/spinal bulbar muscular atrophy. PLoS One (2010) 1.02
The TRK-fused gene is mutated in hereditary motor and sensory neuropathy with proximal dominant involvement. Am J Hum Genet (2012) 1.02
Paraneoplastic neuropathy. Handb Clin Neurol (2013) 1.01
Clinicopathological features of acute autonomic and sensory neuropathy. Brain (2010) 1.00