Haruhiko Akiyama

Summary

Affiliation: Tokyo Institute of Psychiatry
Country: Japan

Publications

  1. pmc The transcription factor Sox9 has essential roles in successive steps of the chondrocyte differentiation pathway and is required for expression of Sox5 and Sox6
    Haruhiko Akiyama
    Department of Molecular Genetics, The University of Texas M D Anderson Cancer Center, Houston, Texas 77030, USA
    Genes Dev 16:2813-28. 2002
  2. ncbi The amino-terminally truncated forms of amyloid beta-protein in brain macrophages in the ischemic lesions of Alzheimer's disease patients
    H Akiyama
    Tokyo Institute of Psychiatry, Japan
    Neurosci Lett 219:115-8. 1996
  3. ncbi Expression of BRI, the normal precursor of the amyloid protein of familial British dementia, in human brain
    Haruhiko Akiyama
    Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, 156 8585, Tokyo, Japan
    Acta Neuropathol 107:53-8. 2004
  4. ncbi [Abeta, tau and alpha-synuclein and glial cells]
    Haruhiko Akiyama
    Tokyo Institute of Psychiatry, Japan
    Nihon Shinkei Seishin Yakurigaku Zasshi 26:23-31. 2006
  5. ncbi Immunohistochemical localization of neprilysin in the human cerebral cortex: inverse association with vulnerability to amyloid beta-protein (Abeta) deposition
    H Akiyama
    Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, 156 8585, Tokyo, Japan
    Brain Res 902:277-81. 2001
  6. ncbi Occurrence of the diffuse amyloid beta-protein (Abeta) deposits with numerous Abeta-containing glial cells in the cerebral cortex of patients with Alzheimer's disease
    H Akiyama
    Tokyo Institute of Psychiatry, Japan
    Glia 25:324-31. 1999
  7. ncbi Variable deposition of amyloid beta-protein (A beta) with the carboxy-terminus that ends at residue valine40 (A beta 40) in the cerebral cortex of patients with Alzheimer's disease: a double-labeling immunohistochemical study with antibodies specific for
    H Akiyama
    Tokyo Institute of Psychiatry, Japan
    Neurochem Res 22:1499-506. 1997
  8. ncbi Amyotrophic lateral sclerosis with dementia: an autopsy case showing many Bunina bodies, tau-positive neuronal and astrocytic plaque-like pathologies, and pallido-nigral degeneration
    Osamu Yokota
    Department of Neuropathology, Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Tokyo, Japan
    Acta Neuropathol 112:633-45. 2006
  9. doi Clinicopathological characterization of Pick's disease versus frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions
    Osamu Yokota
    Department of Neuropathology, Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, Tokyo, 156 8585, Japan
    Acta Neuropathol 117:429-44. 2009
  10. doi Progressive nonfluent aphasia: a rare clinical subtype of FTLD-TDP in Japan
    Naoya Aoki
    Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan
    Neuropathology 32:272-9. 2012

Collaborators

Detail Information

Publications96

  1. pmc The transcription factor Sox9 has essential roles in successive steps of the chondrocyte differentiation pathway and is required for expression of Sox5 and Sox6
    Haruhiko Akiyama
    Department of Molecular Genetics, The University of Texas M D Anderson Cancer Center, Houston, Texas 77030, USA
    Genes Dev 16:2813-28. 2002
    ..We conclude that Sox9 is required during sequential steps of the chondrocyte differentiation pathway...
  2. ncbi The amino-terminally truncated forms of amyloid beta-protein in brain macrophages in the ischemic lesions of Alzheimer's disease patients
    H Akiyama
    Tokyo Institute of Psychiatry, Japan
    Neurosci Lett 219:115-8. 1996
    ..These data suggest that appropriately stimulated macrophages can phagocytose A beta deposits and that digestion of the N-terminal region is an early consequence of this phagocytosis...
  3. ncbi Expression of BRI, the normal precursor of the amyloid protein of familial British dementia, in human brain
    Haruhiko Akiyama
    Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, 156 8585, Tokyo, Japan
    Acta Neuropathol 107:53-8. 2004
    ....
  4. ncbi [Abeta, tau and alpha-synuclein and glial cells]
    Haruhiko Akiyama
    Tokyo Institute of Psychiatry, Japan
    Nihon Shinkei Seishin Yakurigaku Zasshi 26:23-31. 2006
    ....
  5. ncbi Immunohistochemical localization of neprilysin in the human cerebral cortex: inverse association with vulnerability to amyloid beta-protein (Abeta) deposition
    H Akiyama
    Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, 156 8585, Tokyo, Japan
    Brain Res 902:277-81. 2001
    ..A paucity of neprilysin in the hippocampus and association cortices may contribute to the vulnerability of these areas to Abeta deposition...
  6. ncbi Occurrence of the diffuse amyloid beta-protein (Abeta) deposits with numerous Abeta-containing glial cells in the cerebral cortex of patients with Alzheimer's disease
    H Akiyama
    Tokyo Institute of Psychiatry, Japan
    Glia 25:324-31. 1999
    ..Further analysis of these deposits might provide important clues regarding the accumulation and clearance of Abeta in Alzheimer's disease brain...
  7. ncbi Variable deposition of amyloid beta-protein (A beta) with the carboxy-terminus that ends at residue valine40 (A beta 40) in the cerebral cortex of patients with Alzheimer's disease: a double-labeling immunohistochemical study with antibodies specific for
    H Akiyama
    Tokyo Institute of Psychiatry, Japan
    Neurochem Res 22:1499-506. 1997
    ..Such a diversity of A beta species, both in brain parenchyma and in AA, suggests that multiple C-terminal processing mechanisms occur in the cell types responsible for these deposits...
  8. ncbi Amyotrophic lateral sclerosis with dementia: an autopsy case showing many Bunina bodies, tau-positive neuronal and astrocytic plaque-like pathologies, and pallido-nigral degeneration
    Osamu Yokota
    Department of Neuropathology, Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Tokyo, Japan
    Acta Neuropathol 112:633-45. 2006
    ..Although the pathophysiological relationship between tau pathology and the selective involvement of motor neurons, substantia nigra, and globus pallidus was unclear, we considered that it might be more than coincidental...
  9. doi Clinicopathological characterization of Pick's disease versus frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions
    Osamu Yokota
    Department of Neuropathology, Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, Tokyo, 156 8585, Japan
    Acta Neuropathol 117:429-44. 2009
    ....
  10. doi Progressive nonfluent aphasia: a rare clinical subtype of FTLD-TDP in Japan
    Naoya Aoki
    Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan
    Neuropathology 32:272-9. 2012
    ..A few dystrophic neurites and neuronal intranuclear inclusions were also seen. FTLD-TDP showing PNFA seems to be rare but does exist in Japan, similar to that in other countries...
  11. doi Accumulation of phosphorylated TDP-43 in brains of patients with argyrophilic grain disease
    Hiroshige Fujishiro
    Department of Psychogeriatrics, Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, Tokyo 156 8585, Japan
    Acta Neuropathol 117:151-8. 2009
    ....
  12. doi Clinicopathological characteristics of FTLD-TDP showing corticospinal tract degeneration but lacking lower motor neuron loss
    Zen Kobayashi
    Department of Psychogeriatrics, Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, Tokyo 156 8585, Japan
    J Neurol Sci 298:70-7. 2010
    ..This study clarified that FTLD-MND-P cases have characteristic clinicopathological features distinct from those of FTLD-MND-A...
  13. ncbi Relative paucity of tau accumulation in the small areas with abundant Abeta42-positive capillary amyloid angiopathy within a given cortical region in the brain of patients with Alzheimer pathology
    Kenichi Oshima
    Department of Psychiatry, Tokyo Metropolitan Matsuzawa Hospital, 2 1 1 Kamikitazawa, Setagaya Ku, and Juntendo Medical University, Tokyo, Japan
    Acta Neuropathol 111:510-8. 2006
    ..We suppose that both capCAA and senile plaques indicate high Abeta42 in the neuropil but that only Abeta42 in the form of insoluble deposits in senile plaques promotes tau abnormality...
  14. ncbi TDP-43 is deposited in the Guam parkinsonism-dementia complex brains
    Masato Hasegawa
    Department of Molecular Neurobiology, Tokyo Institute of Psychiatry, Tokyo Metropolitan Organization for Medical Research, 2 1 8 Kamikitazawa, Setagaya Ku, Tokyo 156 8585, Japan
    Brain 130:1386-94. 2007
    ..These results suggest that accumulation of TDP-43 is a common process in certain neurodegenerative disorders, including FTLD-U, ALS and G-PDC...
  15. ncbi Identification of amino-terminally cleaved tau fragments that distinguish progressive supranuclear palsy from corticobasal degeneration
    Tetsuaki Arai
    Department of Psychogeriatrics, Tokyo Institute of Psychiatry, Setagaya Ku, Tokyo, Japan
    Ann Neurol 55:72-9. 2004
    ..Such a biochemical divergence may be related to the neuropathological features of these diseases...
  16. doi Atypical FTLD-FUS associated with ALS-TDP: a case report
    Zen Kobayashi
    Dementia Research Project, Tokyo Metropolitan Institute of Medical Science, 2 1 6 Kamikitazawa, Setagaya Ku, Tokyo 156 8506, Japan
    Neuropathology 33:83-6. 2013
    ..To our knowledge, this is the first report of a case in which FTLD-FUS pathology is of a dystrophic neurites-predominant type and FTLD-FUS is associated with ALS-TDP...
  17. doi Molecular dissection of TDP-43 proteinopathies
    Masato Hasegawa
    Department of Neuropathology and Cell Biology, Tokyo Metropolitan Institute of Medical Science, 2 1 6 Kamikitazawa, Setagaya Ku, Tokyo, 156 8506, Japan
    J Mol Neurosci 45:480-5. 2011
    ....
  18. doi Identification of casein kinase-1 phosphorylation sites on TDP-43
    Fuyuki Kametani
    Department of Molecular Neurobiology, Tokyo Institute of Psychiatry, Tokyo Metropolitan Organization for Medical Research, Tokyo, Japan
    Biochem Biophys Res Commun 382:405-9. 2009
    ..Interestingly, 18 of them were located in the C-terminal glycine-rich region of TDP-43. Our results indicate that CK1-mediated phosphorylation may play a role in the pathogenesis of these diseases...
  19. doi Phosphorylated TDP-43 in Alzheimer's disease and dementia with Lewy bodies
    Tetsuaki Arai
    Department of Psychogeriatrics, Tokyo Institute of Psychiatry, Tokyo Metropolitan Organization for Medical Research, 2 1 8 Kamikitazawa, Setagaya Ku, Tokyo 156 8585, Japan
    Acta Neuropathol 117:125-36. 2009
    ..There may be genetic factors, such as mutations or genetic variants of PGRN underlying the co-occurrence of abnormal deposition of TDP-43, tau and alpha-synuclein...
  20. pmc Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
    Masato Hasegawa
    Department of Molecular Neurobiology, Tokyo Institute of Psychiatry, Tokyo Metropolitan Organization for Medical Research, Kamikitazawa, Setagaya Ku, Tokyo, Japan
    Ann Neurol 64:60-70. 2008
    ..The aim of this study was to identify the phosphorylation sites and responsible kinases, and to clarify the pathological significance of phosphorylation of TDP-43...
  21. doi Frontotemporal lobar degeneration with motor neuron disease showing severe and circumscribed atrophy of anterior temporal lobes
    Hiroya Kuwahara
    Department of Neurology and Neurological Science, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan
    J Neurol Sci 297:92-6. 2010
    ..This case report showed typical features of FTLD-MND in clinical course and TDP-43 pathology with unusual severity and distribution of cerebral atrophy, suggesting a unique manifestation of FTLD-MND...
  22. ncbi Argyrophilic grains are not always argyrophilic--distinction from neurofibrillary tangles of diffuse neurofibrillary tangles with calcification revealed by comparison between Gallyas and Campbell-Switzer methods
    Toshiki Uchihara
    Department of Neuropathology, Tokyo Metropolitan Institute for Neuroscience, 2 6 Musashi dai, Fuchu, 183 8526, Tokyo, Japan
    Acta Neuropathol 110:158-64. 2005
    ..Although explanations for these empirical differences are not yet available, awareness of this clear distinction is potentially of diagnostic and pathological significance...
  23. doi Abnormal phosphorylation of Ser409/410 of TDP-43 in FTLD-U and ALS
    Yuki Inukai
    Department of Molecular Neurobiology, Tokyo Institute of Psychiatry, Tokyo Metropolitan Organization for Medical Research, 2 1 8 Kamikitazawa, Setagaya Ku, Tokyo 156 8585, Japan
    FEBS Lett 582:2899-904. 2008
    ..Analysis of postmortem changes of TDP-43 revealed that the amounts of Sarkosyl-insoluble, urea-soluble full-length TDP-43 and a 35kDa N-terminal fragment increased time-dependently...
  24. ncbi TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
    Tetsuaki Arai
    Department of Psychogeriatrics, Tokyo Institute of Psychiatry, Setagaya Ku, Tokyo 156 8585, Japan
    Biochem Biophys Res Commun 351:602-11. 2006
    ....
  25. doi Phosphorylated and cleaved TDP-43 in ALS, FTLD and other neurodegenerative disorders and in cellular models of TDP-43 proteinopathy
    Tetsuaki Arai
    Department of Psychogeriatrics, Tokyo Institute of Psychiatry, Tokyo Metropolitan Organization for Medical Research, Tokyo, Japan
    Neuropathology 30:170-81. 2010
    ..Understanding the mechanism of phosphorylation and truncation of TDP-43 and aggregate formation may be crucial for clarifying the pathogenesis of TDP-43 proteinopathy and for developing useful therapeutics...
  26. ncbi Pathological features of FTLD-FUS in a Japanese population: analyses of nine cases
    Zen Kobayashi
    Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan Department of Neurology, JA Toride Medical Center, Toride, Ibaraki, Japan
    J Neurol Sci 335:89-95. 2013
    ..Redistribution of FUS from the nucleus to the cytoplasm could be associated with the formation of abnormal FUS aggregates in FTLD-FUS. ..
  27. pmc Methylene blue reduced abnormal tau accumulation in P301L tau transgenic mice
    Masato Hosokawa
    Department of Dementia and Higher Brain Function, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan
    PLoS ONE 7:e52389. 2012
    ..Subsequent results of Western blotting analysis revealed that this agent reduced detergent-insoluble phospho-tau. Methylene blue may have potential as a drug candidate for the treatment of tauopathy...
  28. ncbi Basophilic inclusion body disease and neuronal intermediate filament inclusion disease: a comparative clinicopathological study
    Osamu Yokota
    Department of Neuropathology, Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, Tokyo 156 8585, Japan
    Acta Neuropathol 115:561-75. 2008
    ....
  29. doi Phosphorylated and ubiquitinated TDP-43 pathological inclusions in ALS and FTLD-U are recapitulated in SH-SY5Y cells
    Takashi Nonaka
    Department of Molecular Neurobiology, Tokyo Institute of Psychiatry, Tokyo Metropolitan Organization for Medical Research, 2 1 8 Kamikitazawa, Setagaya ku 156 8585, Tokyo, Japan
    FEBS Lett 583:394-400. 2009
    ..Our results suggest that intracellular localization of TDP-43 and proteasomal function may be involved in inclusion formation and neurodegeneration in TDP-43 proteinopathies...
  30. ncbi Alcoholic cerebellar degeneration: a clinicopathological study of six Japanese autopsy cases and proposed potential progression pattern in the cerebellar lesion
    Osamu Yokota
    Department of Neuropathology, Tokyo Institute of Psychiatry, Japan
    Neuropathology 27:99-113. 2007
    ..In addition, cerebellar symptoms may frequently occur if the anterior superior hemisphere and anterior inferior hemisphere, in addition to the anterior superior vermis, are involved...
  31. ncbi Different immunoreactivities of the microtubule-binding region of tau and its molecular basis in brains from patients with Alzheimer's disease, Pick's disease, progressive supranuclear palsy and corticobasal degeneration
    Tetsuaki Arai
    Department of Psychogeriatrics, Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, 156 8585 Tokyo, Japan
    Acta Neuropathol 105:489-98. 2003
    ..Advanced modification may expose the MBD in brain tissues of AD and PiD. It is suggested that the processing of abnormally accumulated tau characterizes the pathophysiology of each tauopathy...
  32. doi Intractable hiccup caused by medulla oblongata lesions: a study of an autopsy patient with possible neuromyelitis optica
    Zen Kobayashi
    Department of Neurology and Neurological Science, Graduate School, Tokyo Medical and Dental University, Tokyo, 113 8519, Japan
    J Neurol Sci 285:241-5. 2009
    ..Although animal experiments have shown that NTS is a critical structure in the hiccup reflex, we demonstrated for the first time the involvement of the NTS in an autopsy patient with intractable hiccup...
  33. ncbi Neuronal and glial inclusions in frontotemporal dementia with or without motor neuron disease are immunopositive for p62
    Tetsuaki Arai
    Department of Psychogeriatrics, Tokyo Institute of Psychiatry, Setagaya Ku, 156 8585, Tokyo, Japan
    Neurosci Lett 342:41-4. 2003
    ..These results suggest that the degenerative process involves p62 in FTD and that the process takes place not only in neurons but also in glial cells...
  34. ncbi Immunohistochemical study of tau accumulation in early stages of Alzheimer-type neurofibrillary lesions
    Takashi Togo
    Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, 156 8585 Tokyo, Japan
    Acta Neuropathol 107:504-8. 2004
    ..The process of AD and age-associated tau pathology is not tau-isoform-specific, but the ratio of 3R-tau and 4R-tau isoforms involved in the neurofibrillary changes varies and is specific to individual neurons...
  35. doi Prion-like properties of pathological TDP-43 aggregates from diseased brains
    Takashi Nonaka
    Department of Neuropathology and Cell Biology, Tokyo Metropolitan Institute of Medical Science, Setagaya Ku, Tokyo 156 8506, Japan
    Cell Rep 4:124-34. 2013
    ..These results indicate that insoluble TDP-43 has prion-like properties that may play a role in the progression of TDP-43 proteinopathy. ..
  36. ncbi Pathological heterogeneity of the precentral gyrus in Pick's disease: a study of 16 autopsy cases
    Kuniaki Tsuchiya
    Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital, 2 1 1, Kamikitazawa, Setagaya Ku, 156 0057, Tokyo, Japan
    Acta Neuropathol 112:29-42. 2006
    ..In Pick's disease with Pick bodies, obvious involvement of the precentral gyrus and pyramidal tract was not previously noticed. Furthermore, we suggest that pyramidal signs in Pick's disease with Pick bodies have been underestimated...
  37. doi Distribution of cerebello-olivary degeneration in idiopathic late cortical cerebellar atrophy: clinicopathological study of four autopsy cases
    Satoru Ota
    Departoment of Neurology, Tokyo Metropolitan Matsuzawa Hospital, Setagaya Ku, Tokyo, Japan
    Neuropathology 28:43-50. 2008
    ..Furthermore, the distribution of the degeneration in the inferior olivary nucleus depends on the distribution of the cerebellar cortex lesions...
  38. ncbi [Autopsy case of Lewy body dementia associated with abundant argyrophilic grains]
    Kenichi Oshima
    Department of Psychiatry, Tokyo Metropolitan Matsuzawa Hospital, 2 1 1 Kamikitazawa, Setagaya Ku, Tokyo 156 0057, Japan
    No To Shinkei 57:695-700. 2005
    ..This report may contribute to the elucidation of the clinicopathological hallmarks of argyrophilic grain dementia and dementia with Lewy bodies...
  39. ncbi Activation of vascular endothelial cells and perivascular cells by systemic inflammation-an immunohistochemical study of postmortem human brain tissues
    Hirotake Uchikado
    Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, 156 8585, Tokyo, Japan
    Acta Neuropathol 107:341-51. 2004
    ..The results of this study could explain the vulnerability of neurological patients to delirium caused by systemic inflammatory conditions...
  40. ncbi Constant and severe involvement of Betz cells in corticobasal degeneration is not consistent with pyramidal signs: a clinicopathological study of ten autopsy cases
    Kuniaki Tsuchiya
    Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital, 2 1 1, Kamikitazawa, Setagaya Ku, 156 0057 Tokyo, Japan
    Acta Neuropathol 109:353-66. 2005
    ..Constant and severe involvement of the fifth layer of the primary motor cortex, including the Betz cells, has not previously been reported in CBD. We suggest that the pyramidal signs in CBD have been disregarded...
  41. doi Methylene blue and dimebon inhibit aggregation of TDP-43 in cellular models
    Makiko Yamashita
    Department of Molecular Neurobiology, Tokyo Institute of Psychiatry, Tokyo Metropolitan Organization for Medical Reearch, 2 1 8 Kamikitazawa, Setagaya Ku, Tokyo 156 8585, Japan
    FEBS Lett 583:2419-24. 2009
    ..These findings were confirmed by immunoblot analysis. The results indicate that MB and dimebon may be useful for the treatment of ALS, FTLD-U and other TDP-43 proteinopathies...
  42. doi Relationship of phosphorylated alpha-synuclein and tau accumulation to Abeta deposition in the cerebral cortex of dementia with Lewy bodies
    Kimiko Obi
    Department of Neurology, Juntendo University School of Medicine, 2 1 1 Hongo, Bunkyo ku, Tokyo, 113 8421, Japan
    Exp Neurol 210:409-20. 2008
    ..The procession from Abeta to neurite pathology in the cerebral cortex of AD and DLB may be unifiable...
  43. pmc Conditional inactivation of TNFα-converting enzyme in chondrocytes results in an elongated growth plate and shorter long bones
    Kenta Saito
    Department of Orthopedic Surgery, School of Medicine, Keio University, Tokyo, Japan
    PLoS ONE 8:e54853. 2013
    ..Taken together, these data suggest that TACE-EGFR signaling in chondrocytes is involved in the turnover of the growth plate during postnatal development via the transcriptional regulation of osteoprotegerin, Rankl, and Mmp-13...
  44. doi Localization of fused in sarcoma (FUS) protein to the post-synaptic density in the brain
    Naoya Aoki
    Dementia Project, Department of Dementia and Higher Brain Function, Tokyo Metropolitan Institute of Medical Science, 2 1 6 Kamikitazawa, Setagaya Ku, Tokyo, 156 8506, Japan
    Acta Neuropathol 124:383-94. 2012
    ..The increase in Alzheimer's disease (AD) is less remarkable but still significant. The dendritic localization of FUS and its increase in FTLD-TDP and AD may have some implication for the pathophysiology of neurodegenerative diseases...
  45. doi Epitope mapping of antibodies against TDP-43 and detection of protease-resistant fragments of pathological TDP-43 in amyotrophic lateral sclerosis and frontotemporal lobar degeneration
    Hiroshi Tsuji
    Department of Neuropathology and Cell Biology, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan
    Biochem Biophys Res Commun 417:116-21. 2012
    ..The antibodies and methods used in this study will be useful for the characterization of abnormal TDP-43 in human materials, as well as in vitro and animal models for TDP-43 proteinopathies...
  46. doi FALS with Gly72Ser mutation in SOD1 gene: report of a family including the first autopsy case
    Zen Kobayashi
    Department of Psychogeriatrics, Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, Tokyo, 156 8585, Japan
    J Neurol Sci 300:9-13. 2011
    ..Involvement of Onuf's nucleus may be a characteristic pathological feature in FALS with Gly72Ser mutation in the SOD1 gene...
  47. ncbi Coexistence of amyotrophic lateral sclerosis and argyrophilic grain disease: a non-demented autopsy case showing circumscribed temporal atrophy and involvement of the amygdala
    Osamu Yokota
    Department of Neuropathology, Tokyo Institute of Psychiatry, Tokyo, Japan
    Neuropathology 27:539-50. 2007
    ..The present case suggests that (i) ALS and AGD do rarely coexist, and (ii) when ALS patients have severe temporal atrophy, not only ALS with dementia but also concurrent AGD should be considered in the differential diagnosis...
  48. doi Occurrence of basophilic inclusions and FUS-immunoreactive neuronal and glial inclusions in a case of familial amyotrophic lateral sclerosis
    Zen Kobayashi
    Department of Psychogeriatrics, Tokyo Institute of Psychiatry, Tokyo, Japan
    J Neurol Sci 293:6-11. 2010
    ..These findings support the idea that both BIs and FUS-ir structures are pathological hallmarks of a subset of ALS cases...
  49. ncbi Differential diagnosis of amyotrophic lateral sclerosis from Guillain-Barré syndrome by quantitative determination of TDP-43 in cerebrospinal fluid
    Masato Hosokawa
    1Department of Dementia and Higher Brain Function, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan
    Int J Neurosci 124:344-9. 2014
    ..4% and the specificity was 84.6%. Quantitative determination of TDP-43 concentrations in the CSF by sandwich ELISA is a potential laboratory test for differentiating ALS from peripheral motor neuropathies such as GBS. ..
  50. ncbi Frontotemporal lobar degeneration with ubiquitin pathology: an autopsy case presenting with semantic dementia and upper motor neuron signs with a clinical course of 19 years
    Osamu Yokota
    Department of Neuropathology, Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagayaku, Tokyo, 156 8585, Japan
    Acta Neuropathol 112:739-49. 2006
    ..After reviewing previous cases of FTLD-MND with a clinical course of more than 10 years, we also noticed that (3) FTLD-MND cases with a long disease duration often show upper motor neuron-predominant involvement...
  51. ncbi "Forme fruste" of amyotrophic lateral sclerosis with dementia: a report of five autopsy cases without dementia and with ubiquitinated intraneuronal inclusions
    Satoru Ota
    Department of Neurology, Tokyo Metropolitan Matsuzawa Hospital, Tokyo, Japan
    Neuropathology 25:326-35. 2005
    ..We also concluded that there is a "forme fruste" of ALS with dementia showing no overt dementia clinically...
  52. pmc Prion-like spreading of pathological α-synuclein in brain
    Masami Masuda-Suzukake
    Department of Neuropathology and Cell Biology, Tokyo Metropolitan Institute of Medical Science, 2 1 6 Kamikitazawa, Setagaya Ku, Tokyo 156 8506, Japan
    Brain 136:1128-38. 2013
    ..This is a new mouse model of sporadic α-synucleinopathy and should be useful for elucidating progression mechanisms and evaluating disease-modifying therapy...
  53. ncbi [An autopsy case of "progressive supranuclear palsy" without psychiatric or neurological signs]
    Kenichi Oshima
    Department of Psychiatry, Tokyo Metropolitan Matsuzawa Hospital, Department of Psychogeriatrics, Tokyo Institute of Psychiatry, Japan
    No To Shinkei 56:157-61. 2004
    ..Staining by Gallyas-Braak methods disclosed tuft-shaped astrocytes in the caudate nucleus, putamen, and thalamus. To our knowledge, this is the first report of "PSP" without psychiatric or neurological signs...
  54. doi Gray matter lesions in Nasu-Hakola disease: a report on three autopsy cases
    Naoya Aoki
    Tokyo Institute of Psychiatry, Japan
    Neuropathology 31:135-43. 2011
    ..These gray matter pathologies are considered to be responsible for some of the clinical manifestations of the disease, including extrapyramidal symptoms...
  55. ncbi Sporadic amyotrophic lateral sclerosis of long duration mimicking spinal progressive muscular atrophy exists: additional autopsy case with a clinical course of 19 years
    Kuniaki Tsuchiya
    Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital, 2 1 1 Kamikitazawa, Setagaya Ku, Tokyo 156 0057, Japan
    Neuropathology 24:228-35. 2004
    ....
  56. ncbi [An autopsy case of argyrophilic grain dementia with abundant neurofibrillary tangles]
    Kenichi Oshima
    Department of Psychiatry, Tokyo Metropolitan Matsuzawa Hospital, Department of Psychogeriatrics, Tokyo Institute of Psychiatry, Tokyo, Japan
    No To Shinkei 55:133-8. 2003
    ..Sarkosyl-insoluble tau extracted from the temporal lobe consisted predominantly of four-repeat tau isoforms. To our knowledge, this is the first report of argyrophilic grain dementia complicated with tangle only dementia...
  57. ncbi Distribution of cerebral cortical lesions in diffuse neurofibrillary tangles with calcification: a clinicopathological study of four autopsy cases showing prominent parietal lobe involvement
    Kuniaki Tsuchiya
    Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital, Kamikitazawa, Setagaya Ku, Japan
    Acta Neuropathol 110:57-68. 2005
    ....
  58. doi Autopsy case of concurrent Huntington's disease and neurofibromatosis type 1
    Ito Kawakami
    Department of Psychiatry, Graduate School of Medicine, Yokohama City University School of Medicine, Yokohama, Japan Dementia Research Project, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan
    Psychogeriatrics 14:81-6. 2014
    ..This is the third report of a case with concurrent HD and NF1 in the world, and the first study in which occurrence of polyglutamine inclusions was confirmed on post-mortem examination. ..
  59. ncbi [An autopsy case of senile dementia with pathological features of Parkinson's disease]
    Kenichi Oshima
    Department of Psychiatry, Tokyo Metropolitan Matsuzawa Hospital, 2 1 1 Kamikitazawa, Setagaya Ku, Tokyo 156 0057, Japan
    No To Shinkei 56:603-6. 2004
    ..That is, it is compatible with Parkinson's disease. We postulate that the clinical features of Parkinson's disease are more widespread than previously considered...
  60. doi Metastatic CNS lymphoma presenting with periventricular dissemination - MRI and neuropathological findings in an autopsy case
    Zen Kobayashi
    Department of Neurology and Neurological Science, Graduate School, Tokyo Medical and Dental University, Tokyo, 113 8519, Japan
    J Neurol Sci 277:109-13. 2009
    ..We considered that the lymphoma cells entered the ventricular system through the choroid plexus of the lateral ventricle, followed by dissemination of the periventricular parenchyma...
  61. doi Clinicopathological study of early progressive multifocal leukoencephalopathy incidentally found in a schizophrenia patient
    Kenichi Oshima
    Department of Psychiatry, Tokyoo Metropolitan Matsuzawa Hospital, Tokyo, Japan
    Neuropathology 29:684-8. 2009
    ..PML could be considered a short-term disease preceding death, as "incidental PML" in this case. This is a rare autopsy case of early PML occurring in a schizophrenia patient with PML...
  62. ncbi Sporadic amyotrophic lateral sclerosis with circumscribed temporal atrophy: a report of an autopsy case without dementia and with ubiquitinated intraneuronal inclusions
    Kuniaki Tsuchiya
    Department of Laboratory Medicine and Pathology, Metropolitan Matsuzawa Hospital, Tokyo, Japan
    Neuropathology 22:308-16. 2002
    ..We also note the possibility that there is a forme fruste of ALS with dementia showing no overt dementia clinically...
  63. ncbi [An autopsied case of schizophrenic patient who died from heat stroke]
    Kazuhiro Niizato
    Department of Psychiatry, Tokyo Metropolitan Matsuzawa Hospital, 2 1 1 Kamikitazawa, Setagaya Ku, Tokyo 156 0057, Japan
    No To Shinkei 58:418-24. 2006
    ..These findings have not been thus far reported, so this case is thought to be the valuable case on considering the variation of neuropathology of "heat stroke"...
  64. doi Molecular analysis and biochemical classification of TDP-43 proteinopathy
    Hiroshi Tsuji
    Department of Neuropathology and Cell Biology, Tokyo Metropolitan Institute of Medical Science, Tokyo 156 8506, Japan
    Brain 135:3380-91. 2012
    ..These results suggest a new clinicopathological classification of TAR DNA-binding protein 43 proteinopathies based on their molecular properties...
  65. ncbi Occurrence of T cells in the brain of Alzheimer's disease and other neurological diseases
    Takashi Togo
    Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, Tokyo 156 8585, Japan
    J Neuroimmunol 124:83-92. 2002
    ..Antigen-triggered clonal expansion is not likely to take place. Local inflammatory conditions might cause accumulation and activation of T cells in the AD brain...
  66. doi Glial clusters and perineuronal glial satellitosis in the basal ganglia of neurofibromatosis type 1
    Osamu Yokota
    Department of Neuropathology, Tokyo Institute of Psychiatry, 2 1 8 Kamikitazawa, Setagaya Ku, Tokyo, 156 8585, Japan
    Acta Neuropathol 116:57-66. 2008
    ..Given these findings, glial clusters and perineuronal glial satellitosis may be histopathological features of the NF1 brain and are probably associated with altered regulation of astrocyte growth in NF1...
  67. doi Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43
    Takashi Nonaka
    Department of Molecular Neurobiology, Tokyo Metropolitan Organization for Medical Research, Tokyo, Japan
    Hum Mol Genet 18:3353-64. 2009
    ....
  68. ncbi Morphometrical reappraisal of motor neuron system of Pick's disease and amyotrophic lateral sclerosis with dementia
    Kenji Ikeda
    Tokyo Metropolitan Matsuzawa Hospital, 2 1 1 Kamikitazawa, Setagaya Ku, Japan
    Acta Neuropathol 104:21-8. 2002
    ..However, the border between the two group is not always clear and there are patients who can not be definitively classified...
  69. ncbi Frequency and clinicopathological characteristics of alcoholic cerebellar degeneration in Japan: a cross-sectional study of 1,509 postmortems
    Osamu Yokota
    Department of Neuropathology, Tokyo Institute of Psychiatry, Tokyo, Japan
    Acta Neuropathol 112:43-51. 2006
    ..This study confirmed the frequency of asymptomatic cerebellar degeneration in alcoholics, suggesting that early intervention in alcoholism in the subclinical phase is important to prevent the development of cerebellar symptoms...
  70. ncbi Degeneration of the inferior olive in spinocerebellar ataxia 6 may depend on disease duration: report of two autopsy cases and statistical analysis of autopsy cases reported to date
    Kuniaki Tsuchiya
    Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital, Tokyo, Japan
    Neuropathology 25:125-35. 2005
    ..Furthermore, a log-rank test on the two groups disclosed a significant difference (P=0.0450). We postulate that the neuronal loss of the inferior olive in SCA6 may depend on disease duration...
  71. ncbi Silver staining profiles distinguish Pick bodies from neurofibrillary tangles of Alzheimer type: comparison between Gallyas and Campbell-Switzer methods
    Toshiki Uchihara
    Department of Neuropathology, Tokyo Metropolitan Institute for Neuroscience, 2 6 Musashi dai, Fuchu, 183 8526, Tokyo, Japan
    Acta Neuropathol 109:483-9. 2005
    ..Although explanations for these empirical differences are not yet available, awareness of this clear distinction is potentially of diagnostic and pathological relevance...
  72. pmc Conditional inactivation of TACE by a Sox9 promoter leads to osteoporosis and increased granulopoiesis via dysregulation of IL-17 and G-CSF
    Keisuke Horiuchi
    Department of Anti aging Orthopedic Research, Keio University, School of Medicine, Shinjuku ku, Tokyo, Japan
    J Immunol 182:2093-101. 2009
    ....
  73. ncbi Pick-body-like inclusions in corticobasal degeneration differ from Pick bodies in Pick's disease
    Kenji Ikeda
    Tokyo Metropolitan Matsuzawa Hospital, 2 1 1 Kamikitazawa, Setagaya Ku, Tokyo 156 0057, Japan
    Acta Neuropathol 103:115-8. 2002
    ..These results are useful for the argument of an overlap between PiD and CBD as well as discussion of the phenotypic resemblance of PB-like inclusions bearing types of FTDP-17 to Pick's disease...
  74. doi Long-term oral intake of aluminium or zinc does not accelerate Alzheimer pathology in AβPP and AβPP/tau transgenic mice
    Haruhiko Akiyama
    Department of Dementia and Higher Brain Function, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan
    Neuropathology 32:390-7. 2012
    ..Such results do not seem to support the notion that excessive oral intake of Al or Zn is a risk factor for AD...
  75. pmc Ectodomain shedding of FLT3 ligand is mediated by TNF-alpha converting enzyme
    Keisuke Horiuchi
    Department of Anti aging Orthopedic Research, Keio University, School of Medicine, Shinjuku ku, Tokyo, Japan
    J Immunol 182:7408-14. 2009
    ..Considering the involvements of FLT3 and FLT3L in hematopoietic malignancies and stem cell mobilization, the identification of the enzyme involved in FLT3L shedding may have important clinical implications...
  76. doi Brief report: requirement of TACE/ADAM17 for hair follicle bulge niche establishment
    Keisuke Nagao
    Department of Dermatology, Keio University School of Medicine, Shinjuku ku, Tokyo, Japan
    Stem Cells 30:1781-5. 2012
    ..This study provides mechanistic implication for human TACE-deficiency and for hair abnormality caused by EGFR inhibitors...
  77. ncbi Specificity of mechanisms for plaque removal after A beta immunotherapy for Alzheimer disease
    Haruhiko Akiyama
    Nat Med 10:117-8; author reply 118-9. 2004
  78. ncbi The transcription factor Sox9 is degraded by the ubiquitin-proteasome system and stabilized by a mutation in a ubiquitin-target site
    Haruhiko Akiyama
    Department of Molecular Genetics, The University of Texas M D Anderson Cancer Center, Houston, TX 77030, USA
    Matrix Biol 23:499-505. 2005
    ..Our in vitro studies indicate that the ubiquitin-proteasome proteolytic system degrades Sox9 and regulates its transcriptional activity...
  79. ncbi [Argyrophilic grain disease clinically mimicking Parkinson's disease with dementia: report of an autopsy case]
    Hirotake Uchikado
    Department of Psychiatry, Yokohama City University School of Medicine, Japan
    No To Shinkei 56:785-8. 2004
    ..On the basis of the above-mentioned clinicopathological findings, parkinsonism with dementia is considered to be one of the clinical manifestations of AGD...
  80. ncbi 4-repeat tauopathy sharing pathological and biochemical features of corticobasal degeneration and progressive supranuclear palsy
    Omi Katsuse
    Department of Psychiatry, Yokohama City University School of Medicine, 3 9 Fukuura, Kanazawa Ku, 236 0004 Yokohama, Japan
    Acta Neuropathol 106:251-60. 2003
    ..The present case shared tau-related pathological and biochemical features of CBD and PSP. These findings support that CBD and PSP are closely associated disorders having a pathogenesis common to 4R tauopathy...
  81. ncbi Immunohistochemical investigation of neurofibrillary tangles and their tau isoforms in brains of limbic neurofibrillary tangle dementia
    Eizo Iseki
    Department of Psychiatry, Juntendo Tokyo Koto Geriatric Medical Center, Juntendo University School of Medicine, 3 3 20 Shinsuna, Koto ku, Tokyo 136 0075, Japan
    Neurosci Lett 405:29-33. 2006
    ..Furthermore, 4R tau-positive astrocytes and grains were found in several patients, suggesting that LNTD is a form of tauopathy...
  82. ncbi Amino-truncated amyloid beta-peptide (Abeta5-40/42) produced from caspase-cleaved amyloid precursor protein is deposited in Alzheimer's disease brain
    Kazuya Takeda
    Department of Vascular Dementia Research, National Institute for Longevity Sciences, NCGG, Obu, Japan
    FASEB J 18:1755-7. 2004
    ..The data collectively suggest that caspase cleavage of APP leads to increased production and deposition of Abeta5-40/42 in the AD brain, and highlight the significance of amino-truncated Abeta in the pathogenesis of AD...
  83. ncbi Ubiquitin-positive frontotemporal lobar degeneration presenting with progressive Gogi (word-meaning) aphasia. A neuropsychological, radiological and pathological evaluation of a Japanese semantic dementia patient
    Yasuhisa Sakurai
    Department of Neurology, Mitsui Memorial Hospital, 1 Kanda Izumi cho, Chiyoda ku, Tokyo 101 8643, Japan
    J Neurol Sci 250:3-9. 2006
    ..Brain 2000; 123: 267-76.] and Hodges et al. [Hodges, J.R., Davies, R.R., Xuereb, J.H., Casey, B., Broe, M., Bak, T.H., et al. Clinicopathological correlates in frontotemporal dementia. Ann Neurol 2004; 56: 399-406.]...
  84. ncbi Familial British dementia: colocalization of furin and ABri amyloid
    Claudia Schwab
    Kinsmen Laboratory of Neurological Research, University of British Columbia, 2255 Wesbrook Mall, Vancouver, BC, V6T 1Z3, Canada
    Acta Neuropathol 106:278-84. 2003
    ..These data suggest that furin may be involved in producing the pathological fragment of ABriPP in vivo and that inhibition of furin might be a method of treating this disorder...
  85. ncbi Lewy body variant of Alzheimer's disease or cerebral type lewy body disease? Two autopsy cases of presenile onset with minimal involvement of the brainstem
    Osamu Yokota
    Department of Neuropsychiatry, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2 5 1 Shikata cho, Okayama 700 8558, Japan
    Neuropathology 27:21-35. 2007
    ....
  86. ncbi Pick's disease with Pick bodies: an unusual autopsy case showing degeneration of the pontine nucleus, dentate nucleus, Clarke's column, and lower motor neuron
    Tatsuro Oda
    Department of Psychiatry, National Hospital Organization Shimofusa Psychiatric Medical Center, Chiba, Japan
    Neuropathology 27:81-9. 2007
    ..The atypical clinical symptoms and the widespread neuropathological abnormalities observed in this case seem to represent an extremely extended form of PDPB...
  87. pmc Interactions between Sox9 and beta-catenin control chondrocyte differentiation
    Haruhiko Akiyama
    Department of Molecular Genetics, The University of Texas M D Anderson Cancer Center, Houston, TX 77030, USA
    Genes Dev 18:1072-87. 2004
    ..Our results strongly suggest that chondrogenesis is controlled by interactions between Sox9 and the Wnt/beta-catenin signaling pathway...
  88. ncbi [An autopsy case of dementia lacking distinctive histology showing semantic dementia]
    Satoru Ota
    Department of Neurology, Tokyo Metropolitan Matsuzawa Hospital, 2 1 1 Kamikitazawa, Setagaya Ku, Tokyo 156 0057, Japan
    No To Shinkei 57:233-8. 2005
    ..On the basis of meticulous perusal of the literature, we believe that our case is the first autopsy case of DLDH reported in Japan...
  89. ncbi Transforming growth factor-beta1 mediates the effects of low-intensity pulsed ultrasound in chondrocytes
    Shogo Mukai
    Department of Orthopaedic Surgery, Kyoto University, Kyoto, Japan
    Ultrasound Med Biol 31:1713-21. 2005
    ..These results indicate that LIPUS promotes the proliferation and retains the differentiation state of chondrocytes in the aggregate culture and that TGF-beta1 plays an important role in mediating the LIPUS effects in chondrocytes...
  90. ncbi [Sox family regulate chondrogenesis]
    Haruhiko Akiyama
    Department of Orthopaedics, Kyoto University
    Clin Calcium 16:368- 72. 2006
    ..Indeed, mouse genetics approaches clearly indicate that these Sox family transcription factors are essential for chondrogenesis...
  91. ncbi Reticulons RTN3 and RTN4-B/C interact with BACE1 and inhibit its ability to produce amyloid beta-protein
    Kiyoko S Murayama
    Department of Demyelinating Disease and Ageing, National Institute of Neuroscience, NCNP, 4 1 1 Ogawahigashi, Kodaira, Tokyo 187 8502, Japan
    Eur J Neurosci 24:1237-44. 2006
    ..Because RTN3 and RTN4-B/C are substantially expressed in neural tissues, our findings suggest that they play important roles in the regulation of BACE1 function and Abeta production in the brain...
  92. ncbi Expression analysis of actin-related genes as an underlying mechanism for mood disorders
    Noriaki Nakatani
    Laboratory for Molecular Psychiatry, RIKEN Brain Science Institute, Saitama, Japan
    Biochem Biophys Res Commun 352:780-6. 2007
    ..These data suggest that the balance of actin dynamics might be altered towards actin depolymerization in mood disorders...
  93. ncbi The lack of expression of the peripheral benzodiazepine receptor characterises microglial response in anaplastic astrocytomas
    Shigetoshi Takaya
    Radioisotope Research Centre, Kyoto University, Kyoto, Japan
    J Neurooncol 85:95-103. 2007
    ..Our results demonstrated PBR suppression in glioma-infiltrating microglia and suggested that PBR may have a relevant role in modulating the anti-tumour inflammatory response in astrocytic tumours...
  94. ncbi [Autopsy case of meningovascular neurosyphilis associated with Fischer's plaques]
    Kimiko Obi
    Department of Neurology, Juntendo University of Medicine, 2 1 1 Hongo, Bunkyo ku, Tokyo 113 8421, Japan
    Brain Nerve 59:797-803. 2007
    ..Attention has to be paid for the occurrence of atypical neurosyphilis in association with increased immuologically compromised hosts and frequent usage of antibiotics...
  95. ncbi Changes in density of calcium-binding-protein-immunoreactive GABAergic neurons in prefrontal cortex in schizophrenia and bipolar disorder
    Tsutomu Sakai
    Department of Psychiatry and Human Behavior, Gunma University Graduate School of Medicine, Gunma, Japan
    Neuropathology 28:143-50. 2008
    ..These results suggest that alterations in specific GABAergic neurons are present in mental disorders, and that such alterations may reflect the vulnerability toward the disorders...
  96. ncbi Corticobasal degeneration as cause of progressive non-fluent aphasia: clinical, radiological and pathological study of an autopsy case
    Masaki Takao
    Department of Neurology, Mihara Memorial Hospital, Isesaki, Gunma, Japan
    Neuropathology 26:569-78. 2006
    ..Clinical diagnosis of corticobasal degeneration sometimes is difficult in individuals with atypical clinical presentations. More exact clinical and radiological criteria may warrant a diagnosis of corticobasal degeneration...