Published in Rinsho Shinkeigaku on January 01, 2012
17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration. Nat Med (2005) 3.19
Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Neuron (2002) 2.98
Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients. Brain (2002) 2.35
HIV-1 nef suppression by virally encoded microRNA. Retrovirology (2004) 2.05
Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy. Nat Med (2003) 1.73
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
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
Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Hum Mol Genet (2004) 1.52
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
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
Reversible disruption of dynactin 1-mediated retrograde axonal transport in polyglutamine-induced motor neuron degeneration. J Neurosci (2006) 1.25
Physical and functional interaction between Dorfin and Valosin-containing protein that are colocalized in ubiquitylated inclusions in neurodegenerative disorders. J Biol Chem (2004) 1.23
Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity. J Biol Chem (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
The potential of GPNMB as novel neuroprotective factor in amyotrophic lateral sclerosis. Sci Rep (2012) 1.06
Loss of TDP-43 causes age-dependent progressive motor neuron degeneration. Brain (2013) 1.05
Hsp70 and Hsp40 improve neurite outgrowth and suppress intracytoplasmic aggregate formation in cultured neuronal cells expressing mutant SOD1. Brain Res (2002) 1.04
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
Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies. Neurobiol Dis (2011) 0.99
Calcium-sensing receptor ubiquitination and degradation mediated by the E3 ubiquitin ligase dorfin. J Biol Chem (2006) 0.95
Dorfin localizes to Lewy bodies and ubiquitylates synphilin-1. J Biol Chem (2003) 0.95
Differentially expressed genes in sporadic amyotrophic lateral sclerosis spinal cords--screening by molecular indexing and subsequent cDNA microarray analysis. FEBS Lett (2002) 0.90
[Dyskinesia-hyperpyrexia syndrome in a patient with Parkinson's disease: a case report]. Rinsho Shinkeigaku (2015) 0.88
X-Linked inhibitor of apoptosis protein is involved in mutant SOD1-mediated neuronal degeneration. J Neurochem (2002) 0.87
Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis. J Neurochem (2004) 0.86
RE: Plasma phospholipid fatty acids and prostate cancer risk in the SELECT trial. J Natl Cancer Inst (2014) 0.86
Factors other than phytosterols in some vegetable oils affect the survival of SHRSP rats. Food Chem Toxicol (2004) 0.86
Confirmatory double-blind, parallel-group, placebo-controlled study of efficacy and safety of edaravone (MCI-186) in amyotrophic lateral sclerosis patients. Amyotroph Lateral Scler Frontotemporal Degener (2014) 0.85
A sodium channel myotonia due to a novel SCN4A mutation accompanied by acquired autoimmune myasthenia gravis. Neurosci Lett (2012) 0.85
Dynamic aspect of reactive oxygen and nitric oxide in oral cavity. J Clin Biochem Nutr (2008) 0.85
Gene expressions specifically detected in motor neurons (dynactin 1, early growth response 3, acetyl-CoA transporter, death receptor 5, and cyclin C) differentially correlate to pathologic markers in sporadic amyotrophic lateral sclerosis. J Neuropathol Exp Neurol (2007) 0.84
Mutant androgen receptor accumulation in spinal and bulbar muscular atrophy scrotal skin: a pathogenic marker. Ann Neurol (2006) 0.83
Dorfin-CHIP chimeric proteins potently ubiquitylate and degrade familial ALS-related mutant SOD1 proteins and reduce their cellular toxicity. Neurobiol Dis (2006) 0.83
Dorfin ameliorates phenotypes in a transgenic mouse model of amyotrophic lateral sclerosis. J Neurosci Res (2010) 0.82
Intraventricular chordoid meningioma presenting with Castleman disease due to overproduction of interleukin-6. Case report. J Neurosurg (2005) 0.81
Dorfin localizes to the ubiquitylated inclusions in Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and amyotrophic lateral sclerosis. Am J Pathol (2003) 0.81
Gene expression profiling toward understanding of ALS pathogenesis. Ann N Y Acad Sci (2006) 0.81
Demographic features of Japanese patients with sporadic inclusion body myositis: a single-center referral experience. Intern Med (2013) 0.81
c-Jun N-terminal kinase pathway mediates Lactacystin-induced cell death in a neuronal differentiated Neuro2a cell line. Brain Res Mol Brain Res (2002) 0.79
Marine n-3 fatty acids and colorectal cancer: is there a real link? Cancer Epidemiol Biomarkers Prev (2006) 0.79
RNP2 of RNA recognition motif 1 plays a central role in the aberrant modification of TDP-43. PLoS One (2013) 0.79
Serum alkaline phosphatase isoenzymes in SD rats detected by polyacrylamide-gel disk electrophoresis. Toxicol Mech Methods (2012) 0.78
Nonmyelinating Schwann cell involvement with well-preserved unmyelinated axons in Charcot-Marie-Tooth disease type 1A. J Neuropathol Exp Neurol (2007) 0.78
Cancer and all-cause mortalities are lower in the higher total cholesterol groups among general populations. World Rev Nutr Diet (2007) 0.77
Prenatal management of the fetus with lethal malformation: from a study of oligohydramnios sequence. Fetal Pediatr Pathol (2011) 0.77
Omega3 fatty acids effectively prevent coronary heart disease and other late-onset diseases--the excessive linoleic acid syndrome. World Rev Nutr Diet (2007) 0.77
Caspase-1 and -3 mRNAs are differentially upregulated in motor neurons and glial cells in mutant SOD1 transgenic mouse spinal cord: a study using laser microdissection and real-time RT-PCR. Neurochem Res (2003) 0.77
Testosterone-lowering activity of canola and hydrogenated soybean oil in the stroke-prone spontaneously hypertensive rat. J Toxicol Sci (2010) 0.76
Why isn't the causal relationship between linoleic acid and mortalities from coronary heart disease and stroke revealed by clinical studies? World Rev Nutr Diet (2007) 0.76
n-6 Polyunsaturated fatty acids and breast cancer. Nutr Cancer (2003) 0.75
Effect of Obstructive Jaundice and Nitric Oxide on the Profiles of Intestinal Bacterial Flora in Wild and iNOS Mice. J Clin Biochem Nutr (2009) 0.75
Archaeal proteasomes effectively degrade aggregation-prone proteins and reduce cellular toxicities in mammalian cells. J Biol Chem (2006) 0.75
Mitochondrial localization of mutant superoxide dismutase 1 triggers caspase-dependent cell death in a cellular model of familial amyotrophic lateral sclerosis. J Biol Chem (2016) 0.75
Association of high total cholesterol with coronary heart disease mortality differs among subject populations--familial hypercholesterolemia as a key concept. World Rev Nutr Diet (2007) 0.75
Mechanisms by which dietary fats affect coronary heart disease mortality. World Rev Nutr Diet (2007) 0.75
[Autonomic neuropathy as the first presentation of T-cell malignant lymphoma--a case report]. Rinsho Shinkeigaku (2015) 0.75
New directions of lipid nutrition for the primary and secondary prevention of coronary heart disease and other late-onset diseases. World Rev Nutr Diet (2007) 0.75
Cancers common in the USA are stimulated by omega 6 fatty acids and large amounts of animal fats, but suppressed by omega 3 fatty acids and cholesterol. World Rev Nutr Diet (2007) 0.75
Objective measures of the pathology of coronary heart disease. World Rev Nutr Diet (2007) 0.75
[Living situation of the in-home patients suffering from amyotrophic lateral sclerosis in Aichi prefecture, Japan]. Rinsho Shinkeigaku (2012) 0.75
[Exploration of pathogenesis-associated molecules and development of disease models for sporadic ALS]. Rinsho Shinkeigaku (2008) 0.75
Pleiotropic effects of statins in the prevention of coronary heart disease--potential side effects. World Rev Nutr Diet (2007) 0.75
Poly(N-isopropylacrylamide)-coated microwell arrays for construction and recovery of multicellular spheroids. J Biosci Bioeng (2013) 0.75
The cholesterol hypothesis--its basis and its faults. World Rev Nutr Diet (2007) 0.75
Changes in dietary fatty acids and life style as major factors for rapidly increasing inflammatory diseases and elderly-onset diseases. World Rev Nutr Diet (2005) 0.75
Dietary canola and soybean oil fed to SHRSP rat dams differently affect the growth and survival of their male pups. J Nutr (2004) 0.75
[Motor neuron disease and ubiquitin-proteasome system dysfunction]. Tanpakushitsu Kakusan Koso (2006) 0.75
Aplasia cutis congenita and skull defects after exposure to methimazole in utero. Intern Med (2005) 0.75