PubRank

Masashi Aoki

Author PubWeight™ 85.64‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders. Neurology 2014 6.73
2 Mutations of optineurin in amyotrophic lateral sclerosis. Nature 2010 4.54
3 Aquaporin-4 antibody-positive cases beyond current diagnostic criteria for NMO spectrum disorders. Neurology 2013 2.52
4 Efficacy and safety of leuprorelin in patients with spinal and bulbar muscular atrophy (JASMITT study): a multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol 2010 2.33
5 Presence of six different lesion types suggests diverse mechanisms of tissue injury in neuromyelitis optica. Acta Neuropathol 2013 1.79
6 NO production results in suspension-induced muscle atrophy through dislocation of neuronal NOS. J Clin Invest 2007 1.70
7 Suppression of dynamin GTPase decreases α-synuclein uptake by neuronal and oligodendroglial cells: a potent therapeutic target for synucleinopathy. Mol Neurodegener 2012 1.63
8 NEDL1, a novel ubiquitin-protein isopeptide ligase for dishevelled-1, targets mutant superoxide dismutase-1. J Biol Chem 2003 1.30
9 Mutant SOD1 linked to familial amyotrophic lateral sclerosis, but not wild-type SOD1, induces ER stress in COS7 cells and transgenic mice. Biochem Biophys Res Commun 2003 1.28
10 Disease progression of human SOD1 (G93A) transgenic ALS model rats. J Neurosci Res 2006 1.24
11 Severe olfactory dysfunction is a prodromal symptom of dementia associated with Parkinson's disease: a 3 year longitudinal study. Brain 2012 1.21
12 DNA single-strand break repair is impaired in aprataxin-related ataxia. Ann Neurol 2007 1.14
13 Loss of ALS2/Alsin exacerbates motor dysfunction in a SOD1-expressing mouse ALS model by disturbing endolysosomal trafficking. PLoS One 2010 1.12
14 Exome sequencing identifies a novel TTN mutation in a family with hereditary myopathy with early respiratory failure. J Hum Genet 2013 1.08
15 Gender difference in association between polymorphism of serotonin transporter gene regulatory region and anxiety. J Psychosom Res 2006 1.07
16 Anti-N-methyl-D-aspartate receptor encephalitis with multiphasic demyelination. Ann Neurol 2014 1.06
17 The AAA-ATPase VPS4 regulates extracellular secretion and lysosomal targeting of α-synuclein. PLoS One 2011 1.06
18 [A patient with limb girdle muscular dystrophy type 2B (LGMD2B) manifesting cardiomyopathy]. Rinsho Shinkeigaku 2004 1.05
19 HyperCKemia related to the initial and recurrent attacks of neuromyelitis optica. Intern Med 2012 1.00
20 Neurofibromatosis type 1 (NF1) tumor suppressor, neurofibromin, regulates the neuronal differentiation of PC12 cells via its associating protein, CRMP-2. J Biol Chem 2008 0.97
21 Development of a high-throughput microarray-based resequencing system for neurological disorders and its application to molecular genetics of amyotrophic lateral sclerosis. Arch Neurol 2008 0.97
22 FALS with FUS mutation in Japan, with early onset, rapid progress and basophilic inclusion. J Hum Genet 2010 0.96
23 Heterozygous UDP-GlcNAc 2-epimerase and N-acetylmannosamine kinase domain mutations in the GNE gene result in a less severe GNE myopathy phenotype compared to homozygous N-acetylmannosamine kinase domain mutations. J Neurol Sci 2012 0.95
24 Alteration of familial ALS-linked mutant SOD1 solubility with disease progression: its modulation by the proteasome and Hsp70. Biochem Biophys Res Commun 2006 0.95
25 Proteome profiling reveals gender differences in the composition of human serum. Proteomics 2010 0.95
26 An inducer of VGF protects cells against ER stress-induced cell death and prolongs survival in the mutant SOD1 animal models of familial ALS. PLoS One 2010 0.94
27 Clinical features and a mutation with late onset of limb girdle muscular dystrophy 2B. J Neurol Neurosurg Psychiatry 2012 0.93
28 Dysferlinopathy associated with rigid spine syndrome. Neuropathology 2004 0.93
29 Miyoshi myopathy patients with novel 5' splicing donor site mutations showed different dysferlin immunostaining at the sarcolemma. Acta Neuropathol 2002 0.93
30 Appearance of phagocytic microglia adjacent to motoneurons in spinal cord tissue from a presymptomatic transgenic rat model of amyotrophic lateral sclerosis. J Neurosci Res 2010 0.92
31 Two cases of elderly-onset hereditary neuropathy with liability to pressure palsy manifesting bilateral peroneal nerve palsies. Case Rep Neurol 2012 0.91
32 Age at onset influences on wide-ranged clinical features of sporadic amyotrophic lateral sclerosis. J Neurol Sci 2008 0.90
33 Cerebrospinal fluid aquaporin-4 antibody levels in neuromyelitis optica attacks. Ann Neurol 2014 0.90
34 Underediting of GluR2 mRNA, a neuronal death inducing molecular change in sporadic ALS, does not occur in motor neurons in ALS1 or SBMA. Neurosci Res 2005 0.90
35 Neuronal NOS is dislocated during muscle atrophy in amyotrophic lateral sclerosis. J Neurol Sci 2010 0.90
36 Increase in number of sporadic inclusion body myositis (sIBM) in Japan. J Neurol 2011 0.90
37 Motor neuron disease in transgenic mice with an H46R mutant SOD1 gene. J Neuropathol Exp Neurol 2007 0.88
38 Corticotropin-releasing hormone receptor 1 gene variants in irritable bowel syndrome. PLoS One 2012 0.88
39 A dopamine receptor antagonist L-745,870 suppresses microglia activation in spinal cord and mitigates the progression in ALS model mice. Exp Neurol 2008 0.88
40 Human hepatocyte growth factor promotes functional recovery in primates after spinal cord injury. PLoS One 2011 0.88
41 Accumulation of chondroitin sulfate proteoglycans in the microenvironment of spinal motor neurons in amyotrophic lateral sclerosis transgenic rats. J Neurosci Res 2008 0.87
42 An in vitro model for Lewy body-like hyaline inclusion/astrocytic hyaline inclusion: induction by ER stress with an ALS-linked SOD1 mutation. PLoS One 2007 0.87
43 Two cases of lumbosacral myeloradiculitis with anti-aquaporin-4 antibody. Neurology 2012 0.87
44 Redox system expression in the motor neurons in amyotrophic lateral sclerosis (ALS): immunohistochemical studies on sporadic ALS, superoxide dismutase 1 (SOD1)-mutated familial ALS, and SOD1-mutated ALS animal models. Acta Neuropathol 2005 0.86
45 A functional variant in ZNF512B is associated with susceptibility to amyotrophic lateral sclerosis in Japanese. Hum Mol Genet 2011 0.86
46 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
47 Histological evidence of redox system breakdown caused by superoxide dismutase 1 (SOD1) aggregation is common to SOD1-mutated motor neurons in humans and animal models. Acta Neuropathol 2003 0.85
48 Occurrence of basophilic inclusions and FUS-immunoreactive neuronal and glial inclusions in a case of familial amyotrophic lateral sclerosis. J Neurol Sci 2010 0.85
49 Optineurin is co-localized with FUS in basophilic inclusions of ALS with FUS mutation and in basophilic inclusion body disease. Acta Neuropathol 2011 0.85
50 Aquaporin-4 antibody-positive myelitis initially biopsied for suspected spinal cord tumors: diagnostic considerations. Mult Scler 2013 0.83
51 Clinical manifestations in patients with SOS1 mutations range from Noonan syndrome to CFC syndrome. J Hum Genet 2008 0.83
52 Late-onset distal myopathy with rimmed vacuoles without mutation in the GNE or dysferlin genes. Muscle Nerve 2005 0.83
53 Neck weakness is a potent prognostic factor in sporadic amyotrophic lateral sclerosis patients. J Neurol Neurosurg Psychiatry 2013 0.83
54 Increase of complement fragment C5a in cerebrospinal fluid during exacerbation of neuromyelitis optica. J Neuroimmunol 2012 0.83
55 A case of late onset riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency manifesting as recurrent rhabdomyolysis and acute renal failure. Intern Med 2011 0.82
56 Effective viscosity for nematic-liquid-crystal viscosity measurement using a shear horizontal wave. Phys Rev E Stat Nonlin Soft Matter Phys 2010 0.82
57 Expression profiling with progression of dystrophic change in dysferlin-deficient mice (SJL). Neurosci Res 2005 0.82
58 Novel dysferlin mutations and characteristic muscle atrophy in late-onset Miyoshi myopathy. Muscle Nerve 2004 0.82
59 Serotonin transporter gene promoter polymorphism and alexithymia. Neuropsychobiology 2012 0.82
60 Lower motor neuron disease caused by a novel FUS/TLS gene frameshift mutation. J Neurol 2012 0.82
61 Factors affecting longitudinal functional decline and survival in amyotrophic lateral sclerosis patients. Amyotroph Lateral Scler Frontotemporal Degener 2014 0.81
62 FUS/TLS-immunoreactive neuronal and glial cell inclusions increase with disease duration in familial amyotrophic lateral sclerosis with an R521C FUS/TLS mutation. J Neuropathol Exp Neurol 2012 0.81
63 [A case of dysferlinopathy asymptomatic for 10 years after an episode of transient muscle weakness]. Rinsho Shinkeigaku 2012 0.81
64 Distal anterior compartment myopathy with early ankle contractures. Muscle Nerve 2007 0.80
65 Intrathecal delivery of hepatocyte growth factor from amyotrophic lateral sclerosis onset suppresses disease progression in rat amyotrophic lateral sclerosis model. J Neuropathol Exp Neurol 2007 0.80
66 [Case of recurrent encephalomyelitis associated with eosinophilia in CSF]. Rinsho Shinkeigaku 2012 0.80
67 Neutralizing antibodies are associated with a reduction of interferon-β efficacy during the treatment of Japanese multiple sclerosis patients. Tohoku J Exp Med 2012 0.80
68 Continuous administration of poloxamer 188 reduces overload-induced muscular atrophy in dysferlin-deficient SJL mice. Neurosci Res 2011 0.79
69 Motoneuron degeneration after facial nerve avulsion is exacerbated in presymptomatic transgenic rats expressing human mutant Cu/Zn superoxide dismutase. J Neurosci Res 2005 0.79
70 A case of dysferlinopathy presenting choreic movements. Mov Disord 2006 0.79
71 Up-regulation of insulin-like growth factor-II receptor in reactive astrocytes in the spinal cord of amyotrophic lateral sclerosis transgenic rats. Tohoku J Exp Med 2008 0.78
72 ITIH4 and Gpx3 are potential biomarkers for amyotrophic lateral sclerosis. J Neurol 2013 0.78
73 Clinical characteristics of four patients with temporal lobe epilepsy associated with elevated anti-GAD antibodies. Rinsho Shinkeigaku 2015 0.78
74 Neuroprotective effect of oxidized galectin-1 in a transgenic mouse model of amyotrophic lateral sclerosis. Exp Neurol 2005 0.78
75 Rapid screening for Japanese dysferlinopathy by fluorescent primer extension. Intern Med 2010 0.77
76 Involvement of activated microglia in increased vulnerability of motoneurons after facial nerve avulsion in presymptomatic amyotrophic lateral sclerosis model rats. Glia 2012 0.77
77 Mitochondrial alterations in transgenic mice with an H46R mutant Cu/Zn superoxide dismutase gene. J Neuropathol Exp Neurol 2009 0.77
78 A case with anti-galactocerebroside antibody-positive Mycoplasma pneumoniae meningoencephalitis presenting secondary hypersomnia. Neurol Sci 2012 0.76
79 Sporadic Inclusion Body Myositis Manifesting as Isolated Muscle Weakness of the Finger Flexors Three Years after Disease Onset. Intern Med 2016 0.75
80 Intractable Hiccup in Demyelinating Disease with Anti-Myelin Oligodendrocyte Glycoprotein (MOG) Antibody. Intern Med 2016 0.75
81 Hypometabolism in the supplementary and anterior cingulate cortices is related to dysphagia in Parkinson's disease: a cross-sectional and 3-year longitudinal cohort study. BMJ Open 2013 0.75
82 FALS with FUS mutation in Japan, with early onset, rapid progress and basophilic inclusion. J Hum Genet 2015 0.75
83 [A case of focal epilepsy manifesting multiple psychiatric auras]. Brain Nerve 2015 0.75
84 [Case report; suspected DPP-4 inhibitor-induced dropped head syndrome]. Nihon Naika Gakkai Zasshi 2013 0.75
85 [Internal medicine and neurological diseases: progress in diagnosis and treatment. Topics: XI. Cancer-associated neurological disorders]. Nihon Naika Gakkai Zasshi 2012 0.75
86 [Therapeutic development for GNE myopathy.] Clin Calcium 2017 0.75
87 [The 41st Scientific Meeting: perspectives of internal medicine; lessons from the disaster of the Great East Japan earthquake: 5. Panel discussion: 1) Medical transportation for patients requiring artificial ventilation]. Nihon Naika Gakkai Zasshi 2014 0.75
88 [Amyotrophic lateral sclerosis (ALS) and fused in sarcoma/translocated in liposarcoma (FUS/TLS)]. Nihon Rinsho 2011 0.75
89 A case of steroid-responsive MADSAM with late appearance of a partial conduction block in the forearm. J Neurol 2014 0.75
90 Late-onset Charcot-Marie-Tooth disease type 1B due to a novel mutation in the extracellular disulfide bridge of MPZ gene. Clin Neurol Neurosurg 2012 0.75
91 An autopsy case involving a 12-year history of amyotrophic lateral sclerosis with CIDP-like polyneuropathy. Intern Med 2014 0.75
92 T2-hyperintensity of the middle cerebellar peduncles in a patient with SCA7. Intern Med 2013 0.75
93 Dorsal-roots enhancement and Wallerian degeneration of dorsal cord in the patient of acute sensory ataxic neuropathy. J Neurol 2009 0.75
94 An autopsy case of a dysferlinopathy patient with cardiac involvement. Muscle Nerve 2012 0.75
95 Questionnaire survey on recruitment for Japanese Neurology Society. Rinsho Shinkeigaku 2016 0.75
96 [Unusual visual impairments in a case of MPO-ANCA associated hypertrophic pachymeningitis]. Rinsho Shinkeigaku 2012 0.75
97 [Pathomechanism and prevalence of sporadic inclusion body myositis (sIBM)]. Rinsho Shinkeigaku 2011 0.75
98 Girdle Sensation Caused by Radiculitis due to Neurosarcoidosis. Intern Med 2015 0.75
99 Anti-voltage-gated potassium channel antibody is associated with chronic autonomic and sensory neuropathy. J Neurol 2012 0.75
100 [Radial nerve palsy as a presenting feature of neuralgic amyotrophy]. Rinsho Shinkeigaku 2013 0.75
101 [Actual state and problems in neurology training at graduate school]. Rinsho Shinkeigaku 2014 0.75
102 [Toxoplasma encephalitis in a patient receiving cyclosporine monotherapy for Behçet disease]. Rinsho Shinkeigaku 2005 0.75
103 [Actual state and problems in neurology training at hospital]. Rinsho Shinkeigaku 2014 0.75
104 Cancer-related Stroke due to Mural Thrombus in the Extracranial Carotid Artery. Intern Med 2016 0.75
105 [Improvement of action myoclonus in a patient with dentatorubral-pallidoluysian atrophy by piracetam]. No To Hattatsu 2004 0.75
106 [Case of Neuro-Behçet disease resembling bacterial meningitis]. Rinsho Shinkeigaku 2008 0.75
107 Brain Metabolic Changes of Cervical Dystonia with Spinocerebellar Ataxia Type 1 after Botulinum Toxin Therapy. Intern Med 2016 0.75
108 Severe symptoms of 16q-ADCA coexisting with SCA8 repeat expansion. J Neurol Sci 2008 0.75
109 Intravenous immunoglobulin treatment successfully improved subacute progressive polyradiculoneuropathy with polyclonal gammopathy. Intern Med 2009 0.75
110 [Dopaminergic drug-induced jaw-opening dystonia in a patient with progressive supranuclear palsy]. Rinsho Shinkeigaku 2013 0.75
111 [Inclusion body myositis]. Brain Nerve 2011 0.75
112 A case of myelitis with anti-aquaporin 4 antibody concomitant with immune thrombocytopenic purpura. Rinsho Shinkeigaku 2014 0.75
113 [Clinical translation of hepatocyte growth factor for amyotrophic lateral sclerosis]. Rinsho Shinkeigaku 2012 0.75
114 [Elevated serum aldolase activity in a patient of non-eosinophilic myofasciitis and synovitis with perifascicular atrophy]. Rinsho Shinkeigaku 2009 0.75
115 [A patient with distal muscular dystrophy without mutations in dysferlin gene but with abnormal dysferlin localization in muscle fibers]. Rinsho Shinkeigaku 2004 0.75
116 [Massive bleeding from tracheoarterial fistula in an amyotrophic lateral sclerosis patient treated with long-term invasive ventilation: an autopsy case report]. Rinsho Shinkeigaku 2008 0.75
117 [Actual state and problems in neurology training in medical schools]. Rinsho Shinkeigaku 2014 0.75
118 Hoarseness due to bilateral vocal cord paralysis as an initial manifestation of familial amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 2005 0.75