PubRank

Manabu Doyu

Author PubWeight™ 45.57‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration. Nat Med 2005 3.19
2 Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Neuron 2002 2.98
3 Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients. Brain 2002 2.35
4 Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy. Nat Med 2003 1.73
5 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
6 Gene expression profile of spinal motor neurons in sporadic amyotrophic lateral sclerosis. Ann Neurol 2005 1.59
7 Disulfide bond mediates aggregation, toxicity, and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD1. J Biol Chem 2007 1.57
8 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
9 Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Hum Mol Genet 2004 1.52
10 Phase 2 trial of leuprorelin in patients with spinal and bulbar muscular atrophy. Ann Neurol 2009 1.43
11 Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease. Proc Natl Acad Sci U S A 2005 1.42
12 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
13 Widespread nuclear and cytoplasmic accumulation of mutant androgen receptor in SBMA patients. Brain 2005 1.33
14 Reversible disruption of dynactin 1-mediated retrograde axonal transport in polyglutamine-induced motor neuron degeneration. J Neurosci 2006 1.25
15 Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity. J Biol Chem 2002 1.19
16 Pathogenesis, animal models and therapeutics in spinal and bulbar muscular atrophy (SBMA). Exp Neurol 2006 1.18
17 Modulation of Hsp90 function in neurodegenerative disorders: a molecular-targeted therapy against disease-causing protein. J Mol Med (Berl) 2006 1.16
18 Hsp70 and Hsp40 improve neurite outgrowth and suppress intracytoplasmic aggregate formation in cultured neuronal cells expressing mutant SOD1. Brain Res 2002 1.04
19 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
20 Dorfin localizes to Lewy bodies and ubiquitylates synphilin-1. J Biol Chem 2003 0.95
21 Differentially expressed genes in sporadic amyotrophic lateral sclerosis spinal cords--screening by molecular indexing and subsequent cDNA microarray analysis. FEBS Lett 2002 0.90
22 [Dyskinesia-hyperpyrexia syndrome in a patient with Parkinson's disease: a case report]. Rinsho Shinkeigaku 2015 0.88
23 X-Linked inhibitor of apoptosis protein is involved in mutant SOD1-mediated neuronal degeneration. J Neurochem 2002 0.87
24 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
25 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
26 A sodium channel myotonia due to a novel SCN4A mutation accompanied by acquired autoimmune myasthenia gravis. Neurosci Lett 2012 0.85
27 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
28 Mutant androgen receptor accumulation in spinal and bulbar muscular atrophy scrotal skin: a pathogenic marker. Ann Neurol 2006 0.83
29 Dorfin-CHIP chimeric proteins potently ubiquitylate and degrade familial ALS-related mutant SOD1 proteins and reduce their cellular toxicity. Neurobiol Dis 2006 0.83
30 Dorfin ameliorates phenotypes in a transgenic mouse model of amyotrophic lateral sclerosis. J Neurosci Res 2010 0.82
31 Demographic features of Japanese patients with sporadic inclusion body myositis: a single-center referral experience. Intern Med 2013 0.81
32 Intraventricular chordoid meningioma presenting with Castleman disease due to overproduction of interleukin-6. Case report. J Neurosurg 2005 0.81
33 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
34 Gene expression profiling toward understanding of ALS pathogenesis. Ann N Y Acad Sci 2006 0.81
35 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
36 Nonmyelinating Schwann cell involvement with well-preserved unmyelinated axons in Charcot-Marie-Tooth disease type 1A. J Neuropathol Exp Neurol 2007 0.78
37 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
38 Archaeal proteasomes effectively degrade aggregation-prone proteins and reduce cellular toxicities in mammalian cells. J Biol Chem 2006 0.75
39 [Autonomic neuropathy as the first presentation of T-cell malignant lymphoma--a case report]. Rinsho Shinkeigaku 2015 0.75
40 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
41 [Living situation of the in-home patients suffering from amyotrophic lateral sclerosis in Aichi prefecture, Japan]. Rinsho Shinkeigaku 2012 0.75
42 [Potent primary leptomeningeal lymphoma masquerading as tuberculous meningitis - a case report]. Rinsho Shinkeigaku 2012 0.75