All Stories

  1. Histopathologic features of an autopsied patient with cerebral small vessel disease and a heterozygous HTRA1 mutation

    Article • Neuropathology, May 2018, Wiley

    Dr Osamu Onodera

  2. Predictors of cognitive impairment in multiple system atrophy

    Article • Journal of the Neurological Sciences, May 2018, Elsevier

    Dr Osamu Onodera

  3. Frequency and characteristics of the TBK1 gene variants in Japanese patients with sporadic amyotrophic lateral sclerosis

    Article • Neurobiology of Aging, April 2018, Elsevier

    Dr Osamu Onodera

  4. Case Report: A patient with spinocerebellar ataxia type 31 and sporadic Creutzfeldt-Jakob disease

    Article • Prion, March 2018, Taylor & Francis

    Dr Osamu Onodera

  5. Expansions of intronic TTTCA and TTTTA repeats in benign adult familial myoclonic epilepsy

    Article • Nature Genetics, March 2018, Springer Science + Business Media

    Dr Osamu Onodera

  6. Loss of Motor Neurons Innervating Cervical Muscles in Patients With Multiple System Atrophy and Dropped Head

    Article • Journal of Neuropathology & Experimental Neurology, February 2018, Oxford University Press (OUP)

    Dr Osamu Onodera

  7. Robustness and Vulnerability of the Autoregulatory System That Maintains Nuclear TDP-43 Levels: A Trade-off Hypothesis for ALS Pathology Based on in Silico Data

    Article • Frontiers in Neuroscience, February 2018, Frontiers

    Dr Osamu Onodera

  8. Operation of a P300-based brain-computer interface in patients with Duchenne muscular dystrophy

    Article • Scientific Reports, January 2018, Nature

    Dr Osamu Onodera

  9. CARASIL families from India with 3 novel null mutations in theHTRA1gene

    Article • Neurology, November 2017, Wolters Kluwer Health

    Dr Osamu Onodera

  10. Diagnostic criteria for adult-onset leukoencephalopathy with axonal spheroids and pigmented glia due to CSF1R mutation

    Article • European Journal of Neurology, October 2017, Wiley

    Dr Osamu Onodera

  11. Apparent diffusion coefficient reduction might be a predictor of poor outcome in patients with posterior reversible encephalopathy syndrome

    Article • Journal of the Neurological Sciences, October 2017, Elsevier

    Dr Osamu Onodera

  12. New diagnostic criteria for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukocencephalopathy in Japan

    Article • Journal of the Neurological Sciences, October 2017, Elsevier

    Dr Osamu Onodera

  13. The Clinical Features, Risk Factors, and Surgical Treatment of Cervicogenic Headache in Patients With Cervical Spine Disorders Requiring Surgery

    Article • Headache The Journal of Head and Face Pain, June 2017, Wiley

    Dr Osamu Onodera, Takayoshi Shimohata

  14. Evidence that phosphorylated ubiquitin signaling is involved in the etiology of Parkinson’s disease

    Article • Human Molecular Genetics, May 2017, Oxford University Press (OUP)

    Dr Osamu Onodera

  15. Microglia preconditioned by oxygen-glucose deprivation promote functional recovery in ischemic rats

    Article • Scientific Reports, February 2017, Springer Science + Business Media

    Dr Osamu Onodera

  16. A CARASIL Patient from Americas with Novel Mutation and Atypical Features: Case Presentation and Literature Review

    Article • Cerebrovascular Diseases, January 2017, Karger Publishers

    Dr Osamu Onodera

  17. Multiple system atrophy: clinicopathological characteristics in Japanese patients

    Article • Proceedings of the Japan Academy Series B, January 2017, Japan Academy

    Dr Osamu Onodera

  18. Dissociation between intact vibratory sensation and impaired joint position sensation may predict ataxia of spinal origin

    Article • Interdisciplinary Neurosurgery, December 2016, Elsevier

    Dr Osamu Onodera

  19. Performance of a real-time PCR–based approach and droplet digital PCR in detecting human parechovirus type 3 RNA

    Article • Journal of Clinical Virology, November 2016, Elsevier

    Dr Osamu Onodera

  20. Clinicopathological characteristics of patients with amyotrophic lateral sclerosis resulting in a totally locked-in state (communication Stage V)

    Article • Acta Neuropathologica Communications, September 2016, Springer Science + Business Media

    Dr Osamu Onodera

  21. Clinical and genetic characterization of adult-onset leukoencephalopathy with axonal spheroids and pigmented glia associated withCSF1Rmutation

    Article • European Journal of Neurology, September 2016, Wiley

    Takuya Konno, Dr Osamu Onodera

  22. Diagnostic Value of Brain Calcifications in Adult-Onset Leukoencephalopathy with Axonal Spheroids and Pigmented Glia

    Article • American Journal of Neuroradiology, September 2016, American Society of Neuroradiology (ASNR)

    Takuya Konno, Dr Osamu Onodera

  23. Characteristic microglial features in patients with hereditary diffuse leukoencephalopathy with spheroids

    Article • Annals of Neurology, September 2016, Wiley

    Takuya Konno, Dr Osamu Onodera

  24. Modifiable Factors Associated with Cognitive Impairment in 1,143 Japanese Outpatients: The Project in Sado for Total Health (PROST)

    Article • Dementia and Geriatric Cognitive Disorders Extra, August 2016, Karger Publishers

    Dr Osamu Onodera

  25. Familial amyotrophic lateral sclerosis with an I104F mutation in the SOD1 gene: Multisystem degeneration with neurofilamentous aggregates and SOD1 inclusions

    Article • Neuropathology, July 2016, Wiley

    Dr Osamu Onodera

  26. Heterogeneity of cerebral TDP-43 pathology in sporadic amyotrophic lateral sclerosis: Evidence for clinico-pathologic subtypes

    Article • Acta Neuropathologica Communications, June 2016, Springer Science + Business Media

    Takuya Konno, Dr Osamu Onodera

  27. Increased cytoplasmicTARDBPmRNA in affected spinal motor neurons in ALS caused by abnormal autoregulation of TDP-43

    Article • Nucleic Acids Research, June 2016, Oxford University Press (OUP)

    Takuya Konno, Dr Osamu Onodera

  28. Distinct molecular mechanisms ofHTRA1mutants in manifesting heterozygotes with CARASIL

    Article • Neurology, April 2016, Wolters Kluwer Health

    Dr Osamu Onodera

  29. Roles of inositol 1,4,5-trisphosphate receptors in spinocerebellar ataxias

    Article • Neurochemistry International, March 2016, Elsevier

    Dr Osamu Onodera

  30. Elevated C-Reactive Protein Is Associated with Cognitive Decline in Outpatients of a General Hospital: The Project in Sado for Total Health (PROST)

    Article • Dementia and Geriatric Cognitive Disorders Extra, January 2016, Karger Publishers

    Dr Osamu Onodera

  31. Association between dialysis treatment and cognitive decline: A study from the Project in Sado for Total Health (PROST), Japan

    Article • Geriatrics and Gerontology International, January 2016, Wiley

    Dr Minako Wakasugi, Dr Osamu Onodera

  32. Characteristic features and progression of abnormalities on MRI for CARASIL

    Article • Neurology, July 2015, Wolters Kluwer Health

    Dr Osamu Onodera

  33. Pathological and Clinical Spectrum of Progressive Supranuclear Palsy: With Special Reference to Astrocytic Tau Pathology

    Article • Brain Pathology, June 2015, Wiley

    Dr Osamu Onodera

  34. Variants associated with Gaucher disease in multiple system atrophy

    Article • Annals of Clinical and Translational Neurology, February 2015, Wiley

    Dr Osamu Onodera

  35. Redefining cerebellar ataxia in degenerative ataxias: lessons from recent research on cerebellar systems

    Article • Journal of Neurology Neurosurgery & Psychiatry, January 2015, BMJ

    Dr Osamu Onodera

  36. C9ORF72repeat-associated non-ATG-translated polypeptides are distributed independently of TDP-43 in a Japanese patient with c9ALS

    Article • Neuropathology and Applied Neurobiology, September 2014, Wiley

    Dr Osamu Onodera, Takuya Konno

  37. A Mutation of COX6A1 Causes a Recessive Axonal or Mixed Form of Charcot-Marie-Tooth Disease

    Article • The American Journal of Human Genetics, September 2014, Elsevier

    Dr Osamu Onodera

  38. Features of Cerebral Autosomal Recessive Arteriopathy With Subcortical Infarcts and Leukoencephalopathy

    Article • Stroke, August 2014, Wolters Kluwer Health

    Dr Osamu Onodera

  39. A 3-year cohort study of the natural history of spinocerebellar ataxia type 6 in Japan

    Article • Orphanet Journal of Rare Diseases, July 2014, Springer Science + Business Media

    Dr Osamu Onodera

  40. Progressive myoclonus epilepsy: extraneuronal brown pigment deposition and system neurodegeneration in the brains of Japanese patients with novelSCARB2mutations

    Article • Neuropathology and Applied Neurobiology, July 2014, Wiley

    Dr Osamu Onodera

  41. A blinded international study on the reliability of genetic testing for GGGGCC-repeat expansions in C9orf72 reveals marked differences in results among 14 laboratories

    Article • Journal of Medical Genetics, April 2014, BMJ

    Dr Osamu Onodera, Takuya Konno

  42. Bunina bodies in motor and non-motor neurons revisited: A pathological study of an ALS patient after long-term survival on a respirator

    Article • Neuropathology, January 2014, Wiley

    Dr Osamu Onodera, Takuya Konno

  43. Aberration of the spliceosome in amyotrophic lateral sclerosis

    Article • Rinsho Shinkeigaku, January 2014, Societas Neurologica Japonica

    Dr Osamu Onodera

  44. Haploinsufficiency of CSF-1R and clinicopathologic characterization in patients with HDLS

    Article • Neurology, December 2013, Wolters Kluwer Health

    Dr Osamu Onodera, Takuya Konno

  45. Early clinical features of patients with progressive supranuclear palsy with predominant cerebellar ataxia

    Article • Parkinsonism & Related Disorders, December 2013, Elsevier

    Dr Osamu Onodera

  46. IP3 Receptors in Neurodegenerative Disorders: Spinocerebellar Ataxias and Huntington’s and Alzheimer’s Diseases

    Article • November 2013, Springer Science + Business Media

    Dr Osamu Onodera

  47. ERBB4 Mutations that Disrupt the Neuregulin-ErbB4 Pathway Cause Amyotrophic Lateral Sclerosis Type 19

    Article • The American Journal of Human Genetics, November 2013, Elsevier

    Dr Osamu Onodera

  48. Minor splicing pathway is not minor any more: Implications for the pathogenesis of motor neuron diseases

    Article • Neuropathology, September 2013, Wiley

    Dr Osamu Onodera

  49. Inhibition of SMG-8, a subunit of SMG-1 kinase, ameliorates nonsense-mediated mRNA decay-exacerbated mutant phenotypes without cytotoxicity

    Article • Proceedings of the National Academy of Sciences, August 2013, Proceedings of the National Academy of Sciences

    Dr Osamu Onodera

  50. Mutations in COQ2 in Familial and Sporadic Multiple-System Atrophy

    Article • New England Journal of Medicine, July 2013, New England Journal of Medicine (NEJM/MMS)

    Dr Osamu Onodera

  51. Sporadic ALS with compound heterozygous mutations in the SQSTM1 gene

    Article • Acta Neuropathologica, June 2013, Springer Science + Business Media

    Dr Osamu Onodera

  52. Decreased number of Gemini of coiled bodies and U12 snRNA level in amyotrophic lateral sclerosis

    Article • Human Molecular Genetics, June 2013, Oxford University Press (OUP)

    Dr Osamu Onodera

  53. Molecular pathogenesis of ALS in TDP43 era

    Article • Rinsho Shinkeigaku, January 2013, Societas Neurologica Japonica

    Dr Osamu Onodera

  54. Japanese amyotrophic lateral sclerosis patients with GGGGCC hexanucleotide repeat expansion in C9ORF72

    Article • Journal of Neurology Neurosurgery & Psychiatry, September 2012, BMJ

    Dr Osamu Onodera, Takuya Konno

  55. Alteration of POLDIP3 Splicing Associated with Loss of Function of TDP-43 in Tissues Affected with ALS

    Article • PLoS ONE, August 2012, PLOS

    Dr Osamu Onodera

  56. Prevalence of inositol 1, 4, 5-triphosphate receptor type 1 gene deletion, the mutation for spinocerebellar ataxia type 15, in Japan screened by gene dosage

    Article • Journal of Human Genetics, February 2012, Nature

    Dr Osamu Onodera

  57. Co-occurrence of argyrophilic grain disease in sporadic amyotrophic lateral sclerosis

    Article • Neuropathology and Applied Neurobiology, January 2012, Wiley

    Dr Osamu Onodera

  58. What is ataxia? -Towards developing a new scale for ataxia

    Article • Rinsho Shinkeigaku, January 2012, Societas Neurologica Japonica

    Dr Osamu Onodera

  59. Primary lateral sclerosis: Upper-motor-predominant amyotrophic lateral sclerosis with frontotemporal lobar degeneration - immunohistochemical and biochemical analyses of TDP-43

    Article • Neuropathology, November 2011, Wiley

    Dr Osamu Onodera

  60. Functional characterization of the P1059L mutation in the inositol 1,4,5-trisphosphate receptor type 1 identified in a Japanese SCA15 family

    Article • Biochemical and Biophysical Research Communications, July 2011, Elsevier

    Dr Osamu Onodera

  61. Genotype-phenotype correlations in early onset ataxia with ocular motor apraxia and hypoalbuminaemia

    Article • Brain, April 2011, Oxford University Press (OUP)

    Dr Osamu Onodera

  62. A novel mutation in the HTRA1 gene causes CARASIL without alopecia

    Article • Neurology, April 2011, Wolters Kluwer Health

    Dr Osamu Onodera

  63. Neuroaxonal integrity evaluated by MR spectroscopy in a case of CARASIL

    Article • Journal of Neurology Neurosurgery & Psychiatry, March 2011, BMJ

    Dr Osamu Onodera

  64. Cerebral small-vessel disease protein HTRA1 controls the amount of TGF- 1 via cleavage of proTGF- 1

    Article • Human Molecular Genetics, February 2011, Oxford University Press (OUP)

    Dr Osamu Onodera

  65. TGF-β family signaling contributes to human cerebral small vessel disease

    Article • Rinsho Shinkeigaku, January 2011, Societas Neurologica Japonica

    Dr Osamu Onodera

  66. What is cerebral small vessel disease?

    Article • Rinsho Shinkeigaku, January 2011, Societas Neurologica Japonica

    Dr Osamu Onodera

  67. 4. Detection of Novel Dementia-related Genes. 2) Dysregulation of TGF-^|^beta; Family Signaling and Hereditary Cerebral Small Vessel Disease: Insight into Molecular Pathogenesis of CARASIL.

    Article • Nihon Naika Gakkai Zasshi, January 2011, Japanese Society of Internal Medicine

    Dr Osamu Onodera

  68. Reduced bowel sounds in Parkinson’s disease and multiple system atrophy patients

    Article • Clinical Autonomic Research, December 2010, Springer Science + Business Media

    Dr Osamu Onodera

  69. Identification of novel SNPs of ABCD1, ABCD2, ABCD3, and ABCD4 genes in patients with X-linked adrenoleukodystrophy (ALD) based on comprehensive resequencing and association studies with ALD phenotypes

    Article • Neurogenetics, July 2010, Springer Science + Business Media

    Dr Osamu Onodera

  70. Long-term disability and prognosis in dentatorubral-pallidoluysian atrophy: A correlation with CAG repeat length

    Article • Movement Disorders, June 2010, Wiley

    Dr Osamu Onodera

  71. Neuroblastoma amplified sequence gene is associated with a novel short stature syndrome characterised by optic nerve atrophy and Pelger-Huet anomaly

    Article • Journal of Medical Genetics, June 2010, BMJ

    Dr Osamu Onodera

  72. The phenotype spectrum of Japanese multiple system atrophy

    Article • Journal of Neurology Neurosurgery & Psychiatry, June 2010, BMJ

    Dr Osamu Onodera

  73. Differential levels of  -synuclein,  -amyloid42 and tau in CSF between patients with dementia with Lewy bodies and Alzheimer's disease

    Article • Journal of Neurology Neurosurgery & Psychiatry, June 2010, BMJ

    Dr Osamu Onodera

  74. Involvement of Onuf’s nucleus in Machado–Joseph disease: a morphometric and immunohistochemical study

    Article • Acta Neuropathologica, May 2010, Springer Science + Business Media

    Dr Osamu Onodera

  75. Polyglutamine Diseases: Where does Toxicity Come from? What is Toxicity? Where are We Going?

    Article • Journal of Molecular Cell Biology, April 2010, Oxford University Press (OUP)

    Dr Osamu Onodera

  76. Alzheimer's disease: Report of two autopsy cases with a clinical diagnosis of corticobasal degeneration

    Article • Neuropathology, April 2010, Wiley

    Dr Osamu Onodera

  77. Sporadic four-repeat tauopathy with frontotemporal lobar degeneration, Parkinsonism, and motor neuron disease: a distinct clinicopathological and biochemical disease entity

    Article • Acta Neuropathologica, February 2010, Springer Science + Business Media

    Dr Osamu Onodera

  78. FTLD/ALS as TDP-43 proteinopathies

    Article • Rinsho Shinkeigaku, January 2010, Societas Neurologica Japonica

    Dr Osamu Onodera

  79. The clinical and pathological spectrum of TDP-43 associated ALS

    Article • Rinsho Shinkeigaku, January 2010, Societas Neurologica Japonica

    Dr Osamu Onodera

  80. Early-Onset Ataxia with Ocular Motor Apraxia and Hypoalbuminemia/Ataxia with Oculomotor Apraxia

    Article • January 2010, Springer Science + Business Media

    Dr Osamu Onodera

  81. Identification of independent APP locus duplication in Japanese patients with early-onset Alzheimer disease

    Article • Journal of Neurology Neurosurgery & Psychiatry, August 2009, BMJ

    Dr Osamu Onodera

  82. Selective occurrence of TDP-43-immunoreactive inclusions in the lower motor neurons in Machado–Joseph disease

    Article • Acta Neuropathologica, June 2009, Springer Science + Business Media

    Dr Osamu Onodera

  83. Depletion of medullary serotonergic neurons in patients with multiple system atrophy who succumbed to sudden death

    Article • Brain, May 2009, Oxford University Press (OUP)

    Dr Osamu Onodera

  84. Novel GFAP mutation in patient with adult-onset Alexander disease presenting with spastic ataxia

    Article • Movement Disorders, May 2009, Wiley

    Dr Osamu Onodera

  85. Association of HTRA1 Mutations and Familial Ischemic Cerebral Small-Vessel Disease

    Article • New England Journal of Medicine, April 2009, New England Journal of Medicine (NEJM/MMS)

    Dr Osamu Onodera

  86. Depression and psychiatric symptoms preceding onset of dementia in a family with early-onset Alzheimer disease with a novel PSEN1 mutation

    Article • Deutsche Zeitschrift für Nervenheilkunde, March 2009, Springer Science + Business Media

    Dr Osamu Onodera

  87. A patient with anti-aquaporin 4 antibody who presented with recurrent hypersomnia, reduced orexin (hypocretin) level, and symmetrical hypothalamic lesions

    Article • Sleep Medicine, February 2009, Elsevier

    Dr Osamu Onodera

  88. 日本神経学会２００８年度学会賞受賞者招待講演 脊髄小脳変性症の分子病態機序の解明

    Article • Rinsho Shinkeigaku, January 2009, Societas Neurologica Japonica

    Dr Osamu Onodera

  89. 脊髄小脳変性症の分子病態機序の解明

    Article • Rinsho Shinkeigaku, January 2009, Societas Neurologica Japonica

    Dr Osamu Onodera

  90. Electroclinical features of epilepsy in patients with juvenile type dentatorubral-pallidoluysian atrophy

    Article • Epilepsia, December 2008, Wiley

    Dr Osamu Onodera

  91. Severe neurological phenotypes of Q129 DRPLA transgenic mice serendipitously created by en masse expansion of CAG repeats in Q76 DRPLA mice

    Article • Human Molecular Genetics, November 2008, Oxford University Press (OUP)

    Dr Osamu Onodera

  92. Sporadic amyotrophic lateral sclerosis of long duration is associated with relatively mild TDP-43 pathology

    Article • Acta Neuropathologica, October 2008, Springer Science + Business Media

    Dr Osamu Onodera

  93. Marinesco-Sjögren syndrome with atrophy of the brain stem tegmentum and dysplastic cytoarchitecture in the cerebral cortex

    Article • Neuropathology, October 2008, Wiley

    Dr Osamu Onodera

  94. Development of a High-Throughput Microarray-Based Resequencing System for Neurological Disorders and Its Application to Molecular Genetics of Amyotrophic Lateral Sclerosis

    Article • Archives of Neurology, October 2008, American Medical Association (AMA)

    Dr Osamu Onodera

  95. Cardiac sympathetic denervation in Parkinson’s disease linked to SNCA duplication

    Article • Acta Neuropathologica, August 2008, Springer Science + Business Media

    Dr Osamu Onodera

  96. Total deletion and a missense mutation of ITPR1 in Japanese SCA15 families

    Article • Neurology, June 2008, Wolters Kluwer Health

    Dr Osamu Onodera

  97. Sporadic amyotrophic lateral sclerosis: two pathological patterns shown by analysis of distribution of TDP-43-immunoreactive neuronal and glial cytoplasmic inclusions

    Article • Acta Neuropathologica, May 2008, Springer Science + Business Media

    Dr Osamu Onodera

  98. Sporadic ataxias in Japan – a population-based epidemiological study

    Article • The Cerebellum, April 2008, Springer Science + Business Media

    Dr Osamu Onodera

  99. TDP-43mutation in familial amyotrophic lateral sclerosis

    Article • Annals of Neurology, April 2008, Wiley

    Dr Osamu Onodera

  100. Patients Homozygous and Heterozygous for SNCA Duplication in a Family With Parkinsonism and Dementia

    Article • Archives of Neurology, April 2008, American Medical Association (AMA)

    Dr Osamu Onodera

  101. Prevalence and incidence rates of chronic inflammatory demyelinating polyneuropathy in the Japanese population

    Article • Journal of Neurology Neurosurgery & Psychiatry, February 2008, BMJ

    Dr Osamu Onodera

  102. Mutational Analysis in Early-Onset Familial Dementia in the Japanese Population

    Article • Dementia and Geriatric Cognitive Disorders, January 2008, Karger Publishers

    Dr Osamu Onodera

  103. Enhanced Accumulation of Phosphorylated  -Synuclein and Elevated -Amyloid 42/40 Ratio Caused by Expression of the Presenilin-1  T440 Mutant Associated with Familial Lewy Body Disease and Variant Alzheimer's Disease

    Article • Journal of Neuroscience, November 2007, Society for Neuroscience

    Dr Osamu Onodera

  104. Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic

    Article • Human Molecular Genetics, October 2007, Oxford University Press (OUP)

    Dr Osamu Onodera

  105. Clinical, molecular and histopathological features of short stature syndrome with novel CUL7 mutation in Yakuts: new population isolate in Asia

    Article • Journal of Medical Genetics, September 2007, BMJ

    Dr Osamu Onodera

  106. Novel locus for benign hereditary chorea with adult onset maps to chromosome 8q21.3 q23.3

    Article • Brain, September 2007, Oxford University Press (OUP)

    Dr Osamu Onodera

  107. Generation of intracellular domain of insulin receptor tyrosine kinase by γ-secretase

    Article • Biochemical and Biophysical Research Communications, August 2007, Elsevier

    Dr Osamu Onodera

  108. Daytime Hypoxemia, Sleep-Disordered Breathing, and Laryngopharyngeal Findings in Multiple System Atrophy

    Article • Archives of Neurology, June 2007, American Medical Association (AMA)

    Dr Osamu Onodera

  109. Aprataxin, causative gene product for EAOH/AOA1, repairs DNA single-strand breaks with damaged 3'-phosphate and 3'-phosphoglycolate ends

    Article • Nucleic Acids Research, May 2007, Oxford University Press (OUP)

    Dr Osamu Onodera

  110. Multiplex Families With Multiple System Atrophy

    Article • Archives of Neurology, April 2007, American Medical Association (AMA)

    Dr Osamu Onodera

  111. TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation

    Article • Acta Neuropathologica, February 2007, Springer Science + Business Media

    Dr Osamu Onodera

  112. Early Development of Autonomic Dysfunction May Predict Poor Prognosis in Patients With Multiple System Atrophy

    Article • Archives of Neurology, February 2007, American Medical Association (AMA)

    Dr Osamu Onodera

  113. Sacsin-related ataxia with neither retinal hypermyelination nor spasticity

    Article • Movement Disorders, January 2007, Wiley

    Dr Osamu Onodera

  114. New mutation in the non-gigantic exon ofSACS in Japanese siblings

    Article • Movement Disorders, January 2007, Wiley

    Dr Osamu Onodera

  115. Clinical and genetic characterizations of 16q-linked autosomal dominant spinocerebellar ataxia (AD-SCA) and frequency analysis of AD-SCA in the Japanese population

    Article • Movement Disorders, January 2007, Wiley

    Dr Osamu Onodera

  116. Botulinum toxin A injections improve apraxia of eyelid opening without overt blepharospasm associated with neurodegenerative diseases

    Article • Movement Disorders, January 2007, Wiley

    Dr Osamu Onodera

  117. Novel Mutation in EIF2B Gene in a Case of Adult-Onset Leukoencephalopathy with Vanishing White Matter

    Article • European Neurology, November 2006, Karger Publishers

    Dr Osamu Onodera

  118. Familial amyotrophic lateral sclerosis: a SOD1-unrelated Japanese family of bulbar type with Bunina bodies and ubiquitin-positive skein-like inclusions in lower motor neurons

    Article • Acta Neuropathologica, October 2006, Springer Science + Business Media

    Dr Osamu Onodera

  119. Spinocerebellar ataxia with ocular motor apraxia and DNA repair

    Article • Neuropathology, August 2006, Wiley

    Dr Osamu Onodera

  120. Long-term therapeutic efficacy and safety of low-dose tacrolimus (FK506) for myasthenia gravis

    Article • Journal of the Neurological Sciences, August 2006, Elsevier

    Dr Osamu Onodera

  121. New HSN2 mutation in Japanese patient with hereditary sensory and autonomic neuropathy type 2

    Article • Neurology, April 2006, Wolters Kluwer Health

    Dr Osamu Onodera

  122. Selective silencing of a mutant transthyretin allele by small interfering RNAs

    Article • Biochemical and Biophysical Research Communications, November 2005, Elsevier

    Dr Osamu Onodera

  123. Natural history of X-linked adrenoleukodystrophy in Japan

    Article • Brain and Development, August 2005, Elsevier

    Dr Osamu Onodera

  124. Age associated axonal features in HNPP with 17p11.2 deletion in Japan

    Article • Journal of Neurology Neurosurgery & Psychiatry, August 2005, BMJ

    Dr Osamu Onodera

  125. Clinical and electrophysiologic correlates of IVIg responsiveness in CIDP

    Article • Neurology, April 2005, Wolters Kluwer Health

    Dr Osamu Onodera

  126. Polyglutamine represses cAMP-responsive-element-mediated transcription without aggregate formation

    Article • Neuroreport, February 2005, Wolters Kluwer Health

    Dr Osamu Onodera

  127. Sacsin-related autosomal recessive ataxia without prominent retinal myelinated fibers in Japan

    Article • Movement Disorders, January 2005, Wiley

    Dr Osamu Onodera

  128. A mutantPSEN1 causes dementia with lewy bodies and variant Alzheimer's disease

    Article • Annals of Neurology, January 2005, Wiley

    Dr Osamu Onodera

  129. Churg-Strauss syndrome and the leukotriene receptor antagonist pranlukast

    Article • Clinical Rheumatology, December 2004, Springer Science + Business Media

    Dr Osamu Onodera

  130. The FHA domain of aprataxin interacts with the C-terminal region of XRCC1

    Article • Biochemical and Biophysical Research Communications, December 2004, Elsevier

    Dr Osamu Onodera

  131.  -Synuclein gene alterations in dementia with Lewy bodies

    Article • Neurology, September 2004, Wolters Kluwer Health

    Dr Osamu Onodera

  132. Quantitative evaluation of brainstem involvement in multiple system atrophy by diffusion-weighted MR imaging

    Article • Deutsche Zeitschrift für Nervenheilkunde, September 2004, Springer Science + Business Media

    Dr Osamu Onodera

  133. Five year follow up of a patient with spinal and bulbar muscular atrophy treated with leuprorelin

    Article • Journal of Neurology Neurosurgery & Psychiatry, August 2004, BMJ

    Dr Osamu Onodera

  134. Adult-onset leukoencephalopathy with vanishing white matter with a missense mutation in EIF2B5

    Article • Neurology, May 2004, Wolters Kluwer Health

    Dr Osamu Onodera

  135. Disruption of the toxic conformation of the expanded polyglutamine stretch leads to suppression of aggregate formation and cytotoxicity

    Article • Biochemical and Biophysical Research Communications, May 2004, Elsevier

    Dr Osamu Onodera

  136. Aprataxin, a novel protein that protects against genotoxic stress

    Article • Human Molecular Genetics, March 2004, Oxford University Press (OUP)

    Dr Osamu Onodera

  137. SCA17 homozygote showing Huntington's disease-like phenotype

    Article • Annals of Neurology, January 2004, Wiley

    Dr Osamu Onodera

  138. Aprataxin, the causative protein for EAOH is a nuclear protein with a potential role as a DNA repair protein

    Article • Annals of Neurology, January 2004, Wiley

    Dr Osamu Onodera

  139. Severe generalized dystonia as a presentation of a patient withaprataxin gene mutation

    Article • Movement Disorders, October 2003, Wiley

    Dr Osamu Onodera

  140. Demyelinating and axonal features of Charcot-Marie-Tooth disease with mutations of myelin-related proteins (PMP22, MPZ and Cx32): a clinicopathological study of 205 Japanese patients

    Article • Brain, January 2003, Oxford University Press (OUP)

    Dr Osamu Onodera, Professor Kazuhiko Watabe

  141. Article • Journal of Human Genetics, November 2002, Nature

    Dr Osamu Onodera

  142. Time course of polyglutamine aggregate body formation and cell death: Enhanced growth in nucleus and an interval for cell death

    Article • Journal of Neuroscience Research, May 2002, Wiley

    Dr Osamu Onodera

  143. Japanese cases of familial hemiplegic migraine with cerebellar ataxia carrying a T666M mutation in the CACNA1A gene

    Article • Journal of Neurology Neurosurgery & Psychiatry, May 2002, BMJ

    Dr Osamu Onodera

  144. Expanded polyglutamine stretches form an ‘aggresome’

    Article • Neuroscience Letters, May 2002, Elsevier

    Dr Osamu Onodera

  145. Interferon beta-1a treatment of corticosteroid sensitive polymyositis

    Article • Journal of Neurology Neurosurgery & Psychiatry, May 2002, BMJ

    Dr Osamu Onodera

  146. Paraneoplastic striatal encephalitis

    Article • Neurology, December 2001, Wolters Kluwer Health

    Dr Osamu Onodera

  147. Article • Nature Genetics, October 2001, Nature

    Dr Osamu Onodera

  148. Amino acid sequences flanking polyglutamine stretches influence their potential for aggregate formation

    Article • Neuroreport, October 2001, Wolters Kluwer Health

    Dr Osamu Onodera

  149. Interaction of expanded polyglutamine stretches with nuclear transcription factors leads to aberrant transcriptional regulation in polyglutamine diseases

    Article • Neuropathology, December 2000, Wiley

    Dr Osamu Onodera

  150. Article • Nature Genetics, September 2000, Nature

    Dr Osamu Onodera

  151. Mutational Analysis of X-Linked Adrenoleukodystrophy Gene

    Article • Cell Biochemistry and Biophysics, January 2000, Springer Science + Business Media

    Dr Osamu Onodera

  152. Polyglutamine Domain Proteins with Expanded Repeats Bind Neurofilament, Altering the Neurofilament Network

    Article • Annals of the New York Academy of Sciences, November 1999, Wiley

    Dr Osamu Onodera

  153. No mutation in the entire coding region of the α-synuclein gene in pathologically confirmed cases of multiple system atrophy

    Article • Neuroscience Letters, July 1999, Elsevier

    Dr Osamu Onodera

  154. Mutational Analysis and Genotype-Phenotype Correlation of 29 Unrelated Japanese Patients With X-linked Adrenoleukodystrophy

    Article • Archives of Neurology, March 1999, American Medical Association (AMA)

    Dr Osamu Onodera

  155. Expanded Polyglutamine Domain Proteins Bind Neurofilament and Alter the Neurofilament Network

    Article • Experimental Neurology, February 1999, Elsevier

    Dr Osamu Onodera

  156. Transgenic mice harboring a full-length human mutant DRPLA gene exhibit age-dependent intergenerational and somatic instabilities of CAG repeats comparable with those in DRPLA patients

    Article • Human Molecular Genetics, January 1999, Oxford University Press (OUP)

    Dr Osamu Onodera

  157. A Japanese family with adrenoleukodystrophy with a codon 291 deletion: a clinical, biochemical, pathological, and genetic report

    Article • Journal of the Neurological Sciences, June 1998, Elsevier

    Dr Osamu Onodera

  158. Apolipoprotein E ε4 allele and progression of cortical Lewy body pathology in Parkinson's disease

    Article • Acta Neuropathologica, May 1998, Springer Science + Business Media

    Dr Osamu Onodera

  159. Molecular cloning, structural organization, sequence, chromosomal assignment, and expression of the mouse alpha-N-acetylgalactosaminidase gene

    Article • Gene, May 1998, Elsevier

    Dr Osamu Onodera

  160. Progressive atrophy of cerebellum and brainstem as a function of age and the size of the expanded CAG repeats in theMJD1 gene in Machado-Joseph disease

    Article • Annals of Neurology, March 1998, Wiley

    Dr Osamu Onodera

  161. Pick's disease: selective occurrence of apolipoprotein E-immunoreactive Pick bodies in the limbic system

    Article • Acta Neuropathologica, January 1998, Springer Science + Business Media

    Dr Osamu Onodera

  162. Inhibition of α-ketoglutarate-and pyruvate dehydrogenase complexes in E. coli by a glutathione S-transferase containing a pathological length poly-Q domain: A possible role of energy deficit in neurological diseases associated with poly-Q expansions?

    Article • AGE, January 1998, Springer Science + Business Media

    Dr Osamu Onodera

  163. Atrophy of the cerebellum and brainstem in dentatorubral pallidoluysian atrophy: Influence of CAG repeat size on MRI findings

    Article • Neurology, December 1997, Wolters Kluwer Health

    Dr Osamu Onodera

  164. Transglutaminase-catalyzed inactivation of glyceraldehyde 3-phosphate dehydrogenase and α-ketoglutarate dehydrogenase complex by polyglutamine domains of pathological length

    Article • Proceedings of the National Academy of Sciences, November 1997, Proceedings of the National Academy of Sciences

    Dr Osamu Onodera

  165. Oligomerization of Expanded-Polyglutamine Domain Fluorescent Fusion Proteins in Cultured Mammalian Cells

    Article • Biochemical and Biophysical Research Communications, September 1997, Elsevier

    Dr Osamu Onodera

  166. Molecular Cloning of Murine Homologue Dentatorubral-Pallidoluysian Atrophy (DRPLA) cDNA: Strong Conservation of a Polymorphic CAG Repeat in the Murine Gene

    Article • Genomics, February 1997, Elsevier

    Dr Osamu Onodera

  167. Toxicity of expanded polyglutamine-domain proteins in Escherichia coli

    Article • FEBS Letters, December 1996, Wiley

    Dr Osamu Onodera

  168. Lack of association of very low density lipoprotein receptor gene polymorphism with caucasian Alzheimer's disease

    Article • Annals of Neurology, August 1996, Wiley

    Dr Osamu Onodera

  169. Association study between schizophrenia and dopamine D3 receptor gene polymorphism

    Article • American Journal of Medical Genetics, July 1996, Wiley

    Dr Osamu Onodera

  170. Lack of association between dopamine D2 receptor gene Cys311 variant and schizophrenia

    Article • American Journal of Medical Genetics, April 1996, Wiley

    Dr Osamu Onodera

  171. Lack of association between dopamine D4 receptor gene and schizophrenia

    Article • American Journal of Medical Genetics, December 1995, Wiley

    Dr Osamu Onodera

  172. Genetic association of the very low density lipoprotein (VLDL) receptor gene with sporadic Alzheimer's disease

    Article • Nature Genetics, October 1995, Nature

    Dr Osamu Onodera

  173. Dentatorubral-pallidoluysian atrophy: Clinical features are closely related to unstable expansions of trinucleotide (CAG) repeat

    Article • Annals of Neurology, June 1995, Wiley

    Dr Osamu Onodera

  174. Trial to establish an animal model of paraneoplastic cerebellar degeneration with anti-Yo antibody

    Article • Clinical Neurology and Neurosurgery, February 1995, Elsevier

    Dr Osamu Onodera

  175. Trial to establish an animal model of paraneoplastic cerebellar degeneration with anti-Yo antibody

    Article • Clinical Neurology and Neurosurgery, February 1995, Elsevier

    Dr Osamu Onodera

  176. Long term course of change in anti-Yo antibody content in paraneoplastic cerebellar degeneration.

    Article • Journal of Neurology Neurosurgery & Psychiatry, February 1995, BMJ

    Dr Osamu Onodera

  177. Dentatorubral-pallidoluysian atrophy (DRPLA): Close correlation of CAG repeat expansions with the wide spectrum of clinical presentations and prominent anticipation

    Article • Seminars in Cell Biology, February 1995, Elsevier

    Dr Osamu Onodera

  178. Partial deletions of putative adrenoleukodystrophy (ALD) gene in Japanese ALD patients

    Article • Human Mutation, January 1995, Wiley

    Dr Osamu Onodera

  179. Passive transfer and active immunization with the recombinant leucine-zipper (Yo) protein as an attempt to establish an animal model of paraneoplastic cerebellar degeneration

    Article • Journal of the Neurological Sciences, December 1994, Elsevier

    Dr Osamu Onodera

  180. Trinucleotide repeat length and rate of progression of Huntington's disease

    Article • Annals of Neurology, October 1994, Wiley

    Dr Osamu Onodera

  181. ApoE–ε4 and early–onset Alzheimer's

    Article • Nature Genetics, May 1994, Nature

    Dr Osamu Onodera

  182. Unstable expansion of CAG repeat in hereditary dentatorubral–pallidoluysian atrophy (DRPLA)

    Article • Nature Genetics, January 1994, Nature

    Dr Osamu Onodera

  183. Chromosomal Localization of the ϵ1, ϵ3, and ζ1 Subunit Genes of the Human NMDA Receptor Channel

    Article • Biochemical and Biophysical Research Communications, December 1993, Elsevier

    Dr Osamu Onodera

  184. Absence of linkage disequilibrium at amyloid precursor protein gene locus in Japanese familia Alzheimer's disease with 717Val→Ile mutation

    Article • Neuroscience Letters, November 1993, Elsevier

    Dr Osamu Onodera

  185. The gene for Machado–Joseph disease maps to human chromosome 14q

    Article • Nature Genetics, July 1993, Nature

    Dr Osamu Onodera

  186. Strong correlation between the number of CAG repeats in androgen receptor genes and the clinical onset of features of spinal and bulbar muscular atrophy

    Article • Neurology, December 1992, Wolters Kluwer Health

    Dr Osamu Onodera

  187. Genomic organization of a cDNA (QM) demonstrating an altered mRNA level in nontumorigenic Wilms' microcell hybrid cells and its localization to Xq28

    Article • Human Molecular Genetics, January 1992, Oxford University Press (OUP)

    Dr Osamu Onodera

  188. Rapid diagnosis Of tuberculous meningitis by polymerase chain reaction (PCR)

    Article • Neurology, October 1990, Wolters Kluwer Health

    Dr Osamu Onodera

  189. Atrial standstill after treadmill exercise test and unique response to isoproterenol infusion in recurrent postexercise syncope

    Article • The American Journal of Cardiology, February 1990, Elsevier

    Dr Osamu Onodera

  190. Research into actual conditions and preventive care in periodontal disease - Relationship between questionnaire results and periodontal disease in youth.

    Article • Nihon Shishubyo Gakkai Kaishi (Journal of the Japanese Society of Periodontology), January 1989, Japanese Society of Periodontology

    Dr Osamu Onodera

  191. The study of dentifrice containing Phellodendron amurense extract on periodontal disease. (II). The clinical effects of dentifrice containing Phellodendron amurense extract and anti-inflammatory agents.

    Article • Nihon Shishubyo Gakkai Kaishi (Journal of the Japanese Society of Periodontology), January 1988, Japanese Society of Periodontology

    Dr Osamu Onodera