I Kanazawa

Summary

Affiliation: University of Tokyo
Country: Japan

Publications

  1. ncbi request reprint Dentatorubral-pallidoluysian atrophy or Naito-Oyanagi disease
    I Kanazawa
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Tokyo 113 8655, Japan
    Neurogenetics 2:1-17. 1998
  2. ncbi request reprint How do neurons die in neurodegenerative diseases?
    I Kanazawa
    Dept of Neurology, University of Tokyo Hospital, 113 8655, Tokyo, Japan
    Trends Mol Med 7:339-44. 2001
  3. pmc Molecular pathology of dentatorubral-pallidoluysian atrophy
    I Kanazawa
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Japan
    Philos Trans R Soc Lond B Biol Sci 354:1069-74. 1999
  4. ncbi request reprint PQBP-1, a novel polyglutamine tract-binding protein, inhibits transcription activation by Brn-2 and affects cell survival
    M Waragai
    Group of Molecular Neurobiology, Department of Neurology, Graduate School of Medicine, University of Tokyo, Japan
    Hum Mol Genet 8:977-87. 1999
  5. ncbi request reprint A single motor unit recording technique for studying the differential activation of corticospinal volleys by transcranial magnetic stimulation
    Y Terao
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, 113-8655, Tokyo, Japan
    Brain Res Brain Res Protoc 7:61-7. 2001
  6. ncbi request reprint Interhemispheric interaction between the hand motor areas in patients with cortical myoclonus
    R Hanajima
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, Hongo, Bunkyo-ku, Tokyo 113-8655, Japan
    Clin Neurophysiol 112:623-6. 2001
  7. pmc Linkage-disequilibrium mapping narrows the Fukuyama-type congenital muscular dystrophy (FCMD) candidate region to <100 kb
    T Toda
    Department of Human Genetics, University of Tokyo, Japan
    Am J Hum Genet 59:1313-20. 1996
  8. pmc Interhemispheric facilitation of the hand motor area in humans
    R Hanajima
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, Tokyo, Japan
    J Physiol 531:849-59. 2001
  9. ncbi request reprint Cloning and mapping of ZNF231, a novel brain-specific gene encoding neuronal double zinc finger protein whose expression is enhanced in a neurodegenerative disorder, multiple system atrophy (MSA)
    H Hashida
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Tokyo, Japan
    Genomics 54:50-8. 1998
  10. ncbi request reprint PQBP-1/Npw38, a nuclear protein binding to the polyglutamine tract, interacts with U5-15kD/dim1p via the carboxyl-terminal domain
    M Waragai
    Department of Neurology, Graduate School of Medicine, University of Tokyo, 7 3 1, Hongo, Bunkyo ku, Tokyo, 113 8655, Japan
    Biochem Biophys Res Commun 273:592-5. 2000

Collaborators

Detail Information

Publications83

  1. ncbi request reprint Dentatorubral-pallidoluysian atrophy or Naito-Oyanagi disease
    I Kanazawa
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Tokyo 113 8655, Japan
    Neurogenetics 2:1-17. 1998
    ..The impact of gene analysis of DRPLA on the clinical genetics and neurology are discussed. Moreover, possible mechanism(s) underlying the neuronal cell death in DRPLA are discussed in terms of the molecular pathology...
  2. ncbi request reprint How do neurons die in neurodegenerative diseases?
    I Kanazawa
    Dept of Neurology, University of Tokyo Hospital, 113 8655, Tokyo, Japan
    Trends Mol Med 7:339-44. 2001
    ..Moreover, the elimination of putative triggering and promoting factors involved in neurodegenerative disease might prevent disease progression...
  3. pmc Molecular pathology of dentatorubral-pallidoluysian atrophy
    I Kanazawa
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Japan
    Philos Trans R Soc Lond B Biol Sci 354:1069-74. 1999
    ..Possible mechanism(s) underlying the neuronal cell death in DRPLA are discussed in terms of molecular pathological points of view...
  4. ncbi request reprint PQBP-1, a novel polyglutamine tract-binding protein, inhibits transcription activation by Brn-2 and affects cell survival
    M Waragai
    Group of Molecular Neurobiology, Department of Neurology, Graduate School of Medicine, University of Tokyo, Japan
    Hum Mol Genet 8:977-87. 1999
    ..These results suggest that PQBP-1 mediates important cellular functions under physiological and pathological conditions via its interaction with polyglutamine tracts...
  5. ncbi request reprint A single motor unit recording technique for studying the differential activation of corticospinal volleys by transcranial magnetic stimulation
    Y Terao
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, 113-8655, Tokyo, Japan
    Brain Res Brain Res Protoc 7:61-7. 2001
    ..The obtained results accord well with recent epidural recording studies, which lends support to the validity of this method...
  6. ncbi request reprint Interhemispheric interaction between the hand motor areas in patients with cortical myoclonus
    R Hanajima
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, Hongo, Bunkyo-ku, Tokyo 113-8655, Japan
    Clin Neurophysiol 112:623-6. 2001
    ..We propose that this releases interhemispheric facilitation from powerful surround inhibition. The consequence is a predominant early facilitation between the hemispheres in patients with myoclonus epilepsy...
  7. pmc Linkage-disequilibrium mapping narrows the Fukuyama-type congenital muscular dystrophy (FCMD) candidate region to <100 kb
    T Toda
    Department of Human Genetics, University of Tokyo, Japan
    Am J Hum Genet 59:1313-20. 1996
    ..Thus, the FCMD gene is most likely to lie within a region of <100 kb containing D9S2107...
  8. pmc Interhemispheric facilitation of the hand motor area in humans
    R Hanajima
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, Tokyo, Japan
    J Physiol 531:849-59. 2001
    ..The stronger the conditioning stimulus was, the greater was the amount of inhibition. These results are compatible with surround inhibition at the motor cortex...
  9. ncbi request reprint Cloning and mapping of ZNF231, a novel brain-specific gene encoding neuronal double zinc finger protein whose expression is enhanced in a neurodegenerative disorder, multiple system atrophy (MSA)
    H Hashida
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Tokyo, Japan
    Genomics 54:50-8. 1998
    ..Elevation of ZNF231 expression may be involved in the pathogenesis of multiple system atrophy. The gene for ZNF231 is located on chromosome 3p21...
  10. ncbi request reprint PQBP-1/Npw38, a nuclear protein binding to the polyglutamine tract, interacts with U5-15kD/dim1p via the carboxyl-terminal domain
    M Waragai
    Department of Neurology, Graduate School of Medicine, University of Tokyo, 7 3 1, Hongo, Bunkyo ku, Tokyo, 113 8655, Japan
    Biochem Biophys Res Commun 273:592-5. 2000
    ..This finding suggests physiological functions of PQBP-1 in splicing, cell cycle, and ubiquitination, through which we can speculate the pathological roles of PQBP-1 in triplet repeat diseases...
  11. ncbi request reprint The genomic structure and expression of MJD, the Machado-Joseph disease gene
    Y Ichikawa
    Department of Neurology, Graduate School of Medicine, The University of Tokyo, Japan
    J Hum Genet 46:413-22. 2001
    ..The sequences of these latter clones relative to the MJD gene in B445M7 indicate that there are three alternative splicing sites and eight polyadenylation signals in MJD that are used to generate the differently sized transcripts...
  12. ncbi request reprint Hemispheric lateralization in the cortical motor preparation for human vocalization
    Y Terao
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan
    J Neurosci 21:1600-9. 2001
    ..Our results also suggested involvement of the motor representation of respiratory muscles and also of supplementary motor cortex...
  13. ncbi request reprint An ancient retrotransposal insertion causes Fukuyama-type congenital muscular dystrophy
    K Kobayashi
    Laboratory of Genome Medicine, Human Genome Center, Institute of Medical Science, University of Tokyo, Japan
    Nature 394:388-92. 1998
    ..To our knowledge, FCMD is the first human disease to be caused by an ancient retrotransposal integration...
  14. ncbi request reprint SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein
    K Nakamura
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    Hum Mol Genet 10:1441-8. 2001
    ..We therefore propose that this new disease be called SCA17 (TBP disease)...
  15. ncbi request reprint Different cortical activity in reading of Kanji words, Kana words and Kana nonwords
    Y Sakurai
    Department of Neurology, Mitsui Memorial Hospital, Tokyo, Japan
    Brain Res Cogn Brain Res 9:111-5. 2000
    ....
  16. ncbi request reprint Degeneration of rabbit sensory neurons induced by passive transfer of anti-GD1b antiserum
    S Kusunoki
    Department of Neurology, School of Medicine, University of Tokyo, Japan
    Neurosci Lett 273:33-6. 1999
    ..Anti-GD1b antibody therefore may cause degeneration in rabbit sensory neurons with central axons extending to the dorsal column...
  17. ncbi request reprint Molecular cloning of a novel apoptosis-related gene, human Nap1 (NCKAP1), and its possible relation to Alzheimer disease
    T Suzuki
    Human Genome Center, Institute of Medical Science, University of Tokyo, Tokyo, Japan
    Genomics 63:246-54. 2000
    ..Antisense oligo DNA of human Nap1 transcripts was found to induce apoptosis of neuronal cells. Based on these results, the possible role of human Nap1 in AD is discussed...
  18. ncbi request reprint Cell cycle arrest enhances the in vitro cellular toxicity of the truncated Machado-Joseph disease gene product with an expanded polyglutamine stretch
    T Yoshizawa
    Department of Neurology, Institute of Clinical Medicine, University of Tsukuba, Tsukuba 305 8575, Japan
    Hum Mol Genet 9:69-78. 2000
    ..Mutant ataxin-3 may confer a higher susceptibility to cell death on cells in the G(0)/G(1)phase...
  19. doi request reprint Evaluation of corticospinal tracts in ALS with diffusion tensor MRI and brainstem stimulation
    N K Iwata
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, Japan
    Neurology 70:528-32. 2008
    ....
  20. ncbi request reprint Cortical activation in reading assessed by region of interest-based analysis and statistical parametric mapping
    Y Sakurai
    Department of Neurology, Mitsui Memorial Hospital, 1 Kanda Izumi cho, Chiyoda ku, Tokyo 101 8643, Japan
    Brain Res Brain Res Protoc 6:167-71. 2001
    ..Furthermore, we conducted cognitive subtraction analysis between Kanji and Kana and between Kana words and nonwords and confirmed that Kana words activated the lateral occipital gyri more extensively than Kanji words...
  21. ncbi request reprint Upregulation of the pro-apoptotic BH3-only peptide harakiri in spinal neurons of amyotrophic lateral sclerosis patients
    T Shinoe
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, 113 8655, Tokyo, Japan
    Neurosci Lett 313:153-7. 2001
    ..Heterodimerization of Hrk with Bcl-2 was detected by immunoprecipitation, which suggests the competition of Hrk and anti-apoptotic Bcl-2. These findings suggest that Hrk plays a role in apoptotic events in ALS pathogenesis...
  22. ncbi request reprint Formic acid dissolves aggregates of an N-terminal huntingtin fragment containing an expanded polyglutamine tract: applying to quantification of protein components of the aggregates
    N Hazeki
    CREST, Tokyo, Japan
    Biochem Biophys Res Commun 277:386-93. 2000
    ..These results suggest that other protein components which are degraded by the proteasome, in addition to huntingtin, might be related to the dynamics of polyglutamine protein aggregates...
  23. ncbi request reprint Different complex formations of dentatorubral-pallidoluysian atrophy (DRPLA) protein in human and rat neurons
    I Yazawa
    Department of Neurology, Toranomon Hospital, Tokyo, Japan
    Biochem Biophys Res Commun 253:209-13. 1998
    ..The differences in these characters suggest structural differences and may be related to these proteins functions...
  24. ncbi request reprint Cloning and expression study of the mouse tetrodotoxin-resistant voltage-gated sodium channel alpha subunit NaT/Scn11a
    K Ogata
    Graduate School of Medicine, University of Tokyo, Tokyo, 113 8655, Japan
    Biochem Biophys Res Commun 267:271-7. 2000
    ..c.), around the phase of organogenesis and gonadal differentiation. These findings demonstrate a unique distribution of NaT/Scn11a and suggest some of its roles in the above-mentioned processes...
  25. ncbi request reprint 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
    T Sato
    Department of Neurology, Brain Research Institute, Niigata University, 1 Asahimachi, Niigata 951 8585, Japan
    Hum Mol Genet 8:99-106. 1999
    ....
  26. ncbi request reprint Abundant expression and cytoplasmic aggregations of [alpha]1A voltage-dependent calcium channel protein associated with neurodegeneration in spinocerebellar ataxia type 6
    K Ishikawa
    Department of Neurology, Tokyo Medical and Dental University, Japan
    Hum Mol Genet 8:1185-93. 1999
    ..The present study indicates that the mechanism of neurodegeneration in SCA6 is associated with cytoplasmic aggregations of the [alpha]1A calcium channel protein caused by a small CAG repeat/polyglutamine expansion in CACNA1A...
  27. ncbi request reprint Motor-evoked potentials: unusual findings
    Y Ugawa
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Japan
    Clin Neurophysiol 110:1641-5. 1999
    ..The aims of this study were to present rare findings of motor evoked potentials (MEPs) in 3 patients with spastic paraparesis and to show that careful interpretation is indispensable in experiments done with very high intensity stimulation...
  28. ncbi request reprint A severe case of subacute sarcoid myositis
    H Takuma
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, 113 8655, Tokyo, Japan
    J Neurol Sci 175:140-4. 2000
    ..The characteristic histology of the granuloma confirmed the diagnosis of sarcoidosis. The diffuse muscle pathology was consistent with the patient's severe clinical course...
  29. ncbi request reprint Differential induction of LTP and LTD is not determined solely by instantaneous calcium concentration: an essential involvement of a temporal factor
    T Mizuno
    Department of Neurology, University of Tokyo, Graduate School of Medicine, Tokyo, 113-8605, Japan
    Eur J Neurosci 14:701-8. 2001
    ..Thus, not only the amplitude but also the duration of postsynaptic Ca2+ elevation could be essential factors for differentially inducing LTP or LTD...
  30. ncbi request reprint Guillain-Barré syndrome associated with IgG monospecific to ganglioside GD1b
    T Miyazaki
    Department of Neurology, National Shimosizu Hospital, Chiba, Japan
    Neurology 56:1227-9. 2001
    ..However, more study is needed to substantiate the roles of anti-GD1b IgG antibodies...
  31. ncbi request reprint Widespread expression of alpha-synuclein and tau immunoreactivity in Hallervorden-Spatz syndrome with protracted clinical course
    Y Saito
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, Tokyo, Japan
    J Neurol Sci 177:48-59. 2000
    ....
  32. pmc Japanese families with autosomal dominant pure cerebellar ataxia map to chromosome 19p13.1-p13.2 and are strongly associated with mild CAG expansions in the spinocerebellar ataxia type 6 gene in chromosome 19p13.1
    K Ishikawa
    Department of Neurology, Institute of Clinical Medicine, University of Tsukuba, Japan
    Am J Hum Genet 61:336-46. 1997
    ..We conclude that more than half of Japanese cases of ADPCA map to 19p13.1-p13.2 and are strongly associated with the mild CAG expansion in the SCA6/CACNL1A4 gene...
  33. ncbi request reprint Lambert-Eaton myasthenic syndrome associated with idiopathic thrombocytopenic purpura and diffuse panbronchiolitis: long-term remission after a course of intravenous immunoglobulin combined with low-dose prednisolone
    T Takata
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Japan
    Am J Med Sci 318:353-5. 1999
    ..4 g/kg/day x 5 days), low-dose PSL (20 mg/day) alleviated the LEMS and ITP, and the diseases have remained in remission for 8 months without additional IVIG. We suspect that there is a synergistic relationship between IVIG and PSL...
  34. ncbi request reprint Decreased sensory cortical excitability after 1 Hz rTMS over the ipsilateral primary motor cortex
    H Enomoto
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, Hongo, Bunkyo-ku, 113-8655, Tokyo, Japan
    Clin Neurophysiol 112:2154-8. 2001
    ..1 times the active motor threshold, probably via cortico-cortical pathways between motor and sensory cortex...
  35. ncbi request reprint Distal myopathy with rimmed vacuoles (DMRV): new GNE mutations and splice variant
    H Tomimitsu
    Department of Neurology and Neurological Science, Graduate School, Tokyo Medical and Dental University, University of Tokyo, Japan
    Neurology 62:1607-10. 2004
    ..In addition, a splice-variant characteristic of the skeletal muscle was found, whereas the difference of the expression level between GNE-mutated and -nonmutated patients was not apparent...
  36. ncbi request reprint Cortical activity associated with vocalization and reading proper
    Y Sakurai
    Department of Neurology, Mitsui Memorial Hospital, Tokyo, Japan
    Brain Res Cogn Brain Res 12:161-5. 2001
    ....
  37. ncbi request reprint A unique origin and multistep process for the generation of expanded DRPLA triplet repeats
    H Yanagisawa
    National Children s Medical Research Center, Tokyo, Japan
    Hum Mol Genet 5:373-9. 1996
    ....
  38. ncbi request reprint Anti-Gal-C antibodies in GBS subsequent to mycoplasma infection: evidence of molecular mimicry
    S Kusunoki
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Japan
    Neurology 57:736-8. 2001
    ..pneumoniae. These data show that a Gal-C-like structure is present in M. pneumoniae, indicative of molecular mimicry between a major myelin glycolipid, Gal-C, and M. pneumoniae...
  39. ncbi request reprint Mechanism of short-term memory and repetition in conduction aphasia and related cognitive disorders: a neuropsychological, audiological and neuroimaging study
    Y Sakurai
    Department of Neurology, School of Medicine, University of Tokyo, Japan
    J Neurol Sci 154:182-93. 1998
    ....
  40. ncbi request reprint YAC and cosmid contigs encompassing the Fukuyama-type congenital muscular dystrophy (FCMD) candidate region on 9q31
    M Miyake
    Department of Human Genetics, Graduate School of International Health, University of Tokyo, Japan
    Genomics 40:284-93. 1997
    ..Thus, we have constructed the first high-resolution physical map of the FCMD candidate region. The YAC and cosmid contigs established here will be a crucial resource for identification of the FCMD gene and other genes in this region...
  41. ncbi request reprint Japanese SCA families with an unusual phenotype linked to a locus overlapping with SCA15 locus
    K Hara
    Department of Neurology, Niigata University Brain Research Institute, Japan
    Neurology 62:648-51. 2004
    ..Despite the difference in phenotypes, there remains a possibility that the causative gene for these Japanese SCA is allelic to SCA15...
  42. ncbi request reprint Polar amino acid-rich sequences bind to polyglutamine tracts
    I Imafuku
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Japan
    Biochem Biophys Res Commun 253:16-20. 1998
    ..Three of these clones could form polar helical structures. These observations suggest that polar amino acid-rich sequences are essential for binding to the polyglutamine tract...
  43. ncbi request reprint [Skeletal muscle pathology of chronic graft versus host disease accompanied with myositis, affecting predominantly respiratory and distal muscles, and hemosiderosis]
    Y Oya
    Department of Neurology, University of Tokyo Hospital
    Rinsho Shinkeigaku 41:612-6. 2001
    ..Although we ascribed respiratory failure largely to GVHD myositis, weakness of the lower leg appeared to be aggravated by iron deposition superimposing the underlying GVHD myositis...
  44. pmc Screening for imprinted genes by allelic message display: identification of a paternally expressed gene impact on mouse chromosome 18
    Y Hagiwara
    Human Genome Center, Institute of Medical Science, University of Tokyo, 4 6 1 Shirokane dai, Minato ku, Tokyo 108, Japan
    Proc Natl Acad Sci U S A 94:9249-54. 1997
    ..Hence, AMD would considerably accelerate the identification of imprinted genes playing pivotal roles in mammalian development and the pathogenesis of various diseases...
  45. ncbi request reprint Jugular foramen syndrome caused by varicella zoster virus infection in a patient with ipsilateral hypoplasia of the jugular foramen
    T Hayashi
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, The University of Tokyo, Hongo 7 3 1, Bunkyo ku, Tokyo, Japan
    J Neurol Sci 172:70-2. 2000
    ..Anatomical narrowing of the jugular foramen in this patient may have contributed to entrapment of the affected nerves at their passage through the foramen...
  46. ncbi request reprint Abnormal gene product identified in hereditary dentatorubral-pallidoluysian atrophy (DRPLA) brain
    I Yazawa
    Department of Neurology, Faculty of Medicine, University of Tokyo, Japan
    Nat Genet 10:99-103. 1995
    ....
  47. ncbi request reprint Diphenylpyraline-responsive parkinsonism in cerebrotendinous xanthomatosis: long-term follow up of three patients
    T Ohno
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, Tokyo, Japan
    J Neurol Sci 182:95-7. 2001
    ..The mechanism of the action of DPP on parkinsonism is unclear, however, the drug seems to be a therapeutic choice for treating parkinsonism in CTX...
  48. ncbi request reprint Machado-Joseph disease gene products carrying different carboxyl termini
    J Goto
    Department of Neurology, Institute for Brain Research, Faculty of Medicine, University of Tokyo, Japan
    Neurosci Res 28:373-7. 1997
    ..Genetic analysis results suggest that Japanese MJD mutations are associated with allele A...
  49. ncbi request reprint Stiff-person syndrome associated with invasive thymoma: a case report
    H Hagiwara
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, Hongo 7-3-1, Tokyo, 113-8655, Bunkyo, Japan
    J Neurol Sci 193:59-62. 2001
    ..The present case is the first that associates stiff-person syndrome with invasive thymoma, and not accompanied by MG. The autoimmune mechanism, in this case, may be triggered by the invasive thymoma...
  50. ncbi request reprint Benefit of IVIG for long-standing ataxic sensory neuronopathy with Sjögren's syndrome. IV immunoglobulin
    Y Takahashi
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Japan
    Neurology 60:503-5. 2003
    ..The authors conclude that IVIG is safe and effective to treat even chronically debilitated patients who have the disease, presumably because it ameliorates smoldering inflammation...
  51. ncbi request reprint Hemiparkinsonism associated with a mesencephalic tumor
    M Yoshimura
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, Tokyo, Japan
    J Neurol Sci 197:89-92. 2002
    ..This case is significant due to the paucity of reports regarding the occurrence of a relatively pure parkinsonism that is associated with a mesencephalic space-occupying lesion...
  52. ncbi request reprint Variability in immunohistochemistries of IgM M-proteins binding to sulfated glucuronyl paragloboside
    M Shiina
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, 7-3-1 Hongo, Tokyo 113-8655, Bunkyo, Japan
    J Neuroimmunol 116:206-12. 2001
    ..Only three patients (all in group B) showed some response to the immunotherapies. Weak reactivities to P0 and and PMP-22 might indicate the possibility of improvement after the immunotherapies...
  53. ncbi request reprint Anti-GQ1b antibody as a factor predictive of mechanical ventilation in Guillain-Barré syndrome
    K Kaida
    Third Department of Internal Medicine, National Defense Medical College, Saitama Ken, University of Tokyo, Japan
    Neurology 62:821-4. 2004
    ..04). The presence of the antibody may be a factor predictive of respiratory failure in GBS...
  54. ncbi request reprint Zonisamide has beneficial effects on Parkinson's disease patients
    M Murata
    Department of Neurology, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    Neurosci Res 41:397-9. 2001
    ..Seven clearly showed lessening of symptoms, especially wearing-off. We speculate that long lasting activation of dopamine synthesis by ZNS ameliorates parkinsonian symptoms, in particular wearing-off...
  55. ncbi request reprint Positive symptoms in multiple sclerosis: their treatment with sodium channel blockers, lidocaine and mexiletine
    M Sakurai
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Japan
    J Neurol Sci 162:162-8. 1999
    ..Na channel blockers have a dual effect on symptoms in MS, depending on whether symptoms are positive or negative. The mechanism that produces positive symptoms and the effects of the drugs on these symptoms are discussed...
  56. ncbi request reprint In vitro formation of corticospinal synapses in an organotypic slice co-culture
    H Takuma
    Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, Tokyo 113 8655, Japan
    Neuroscience 109:359-70. 2002
    ..Our results suggest that synapse formation shows preferences along and perpendicular to the neuraxis of the spinal cord...
  57. ncbi request reprint Molecular cloning and expression of a novel truncated form of chicken trkC
    H Okazawa
    Department of Neurology, Faculty of Medicine, University of Tokyo, Japan
    FEBS Lett 329:171-7. 1993
    ..The truncated form is scarcely expressed during the embryonic stages. The conservation of the truncated trkC beyond species suggests they have specific functions...
  58. ncbi request reprint Genetic association of the very low density lipoprotein (VLDL) receptor gene with sporadic Alzheimer's disease
    K Okuizumi
    Department of Neurology, Niigata University, Japan
    Nat Genet 11:207-9. 1995
    ..7 (95% CI = [2.9-25.8]). Our results suggest that the VLDL-R gene is a susceptibility gene for AD...
  59. ncbi request reprint Neurotransmitter abnormality in Rolling mouse Nagoya, an ataxic mutant mouse
    O Muramoto
    Brain Res 215:295-304. 1981
    ....
  60. ncbi request reprint Human genes for KNSL4 and MAZ are located close to one another on chromosome 16p11.2
    J Song
    RIKEN, 3 1 1 Koyadai, Tsukuba, Ibaraki, 305 0074, Japan
    Genomics 52:374-7. 1998
    ..The gene for KNSL4 spanned 16 kb and consisted of 14 exons and 13 introns, while the gene for MAZ spanned 6 kb and consisted of 5 exons and 4 introns. The two genes were mapped to chromosome 16p11.2 by fluorescence in situ hybridization...
  61. ncbi request reprint Genomic organization and expression of a human gene for Myc-associated zinc finger protein (MAZ)
    J Song
    Tsukuba Life Science Center, RIKEN, 3 1 1 Koyadai, Tsukuba, Ibaraki 305, Japan
    J Biol Chem 273:20603-14. 1998
    ..Our results indicate that the 5'-end flanking sequences are responsible for the promoter activities of the MAZ gene...
  62. ncbi request reprint PQBP-1 increases vulnerability to low potassium stress and represses transcription in primary cerebellar neurons
    Y Enokido
    Division of Protein Biosynthesis, Institute for Protein Research, Osaka University, Japan
    Biochem Biophys Res Commun 294:268-71. 2002
    ..Our results indicate that overexpression of PQBP-1 inhibits the basal transcription in cerebellar neurons and increases their vulnerability to low potassium conditions...
  63. ncbi request reprint alpha-Synuclein affects the MAPK pathway and accelerates cell death
    A Iwata
    Laboratory for CAG Repeat Diseases, Molecular Neuropathology Group, RIKEN Brain Science Institute, 2-1 Hirosawa, Wako-shi, Saitama 351-0198, Japan
    J Biol Chem 276:45320-9. 2001
    ..Thus, we conclude that alpha-synuclein regulates the MAPK pathway by reducing the amount of available active MAPK. Our findings suggest a mechanism for pathogenesis and thus offer therapeutic insight into synucleinopathies...
  64. ncbi request reprint Structural organization and expression of the mouse gene for Pur-1, a highly conserved homolog of the human MAZ gene
    J Song
    Tsukuba Life Science Center, Ibaraki, Japan
    Eur J Biochem 259:676-83. 1999
    ..This region also mediated stimulation of basal transcription by ectopically expressed MAZ protein. We conclude that the Pur-1 gene is the murine homolog of human MAZ and, like it, belongs to the family of housekeeping genes...
  65. ncbi request reprint Identification of a novel human voltage-gated sodium channel alpha subunit gene, SCN12A
    S Y Jeong
    CREST, Japan Science and Technology Corporation, Saitama, 332 0012, Japan
    Biochem Biophys Res Commun 267:262-70. 2000
    ..Both neurons and glial cells express SCN12A. SCN12A maps to human chromosome 3p23-p21.3. These results suggest that SCN12A is a tetrodotoxin-resistant (TTX-R) sodium channel expressed in the central nervous system and nonneural tissues...
  66. ncbi request reprint Two consecutive zinc fingers in Sp1 and in MAZ are essential for interactions with cis-elements
    J Song
    RIKEN (The Institute of Physical and Chemical Research, Tsukuba Institute, BioResource Center, 3-1-1 Koyadai, Tsukuba, Ibaraki 305-0074, Japan
    J Biol Chem 276:30429-34. 2001
    ..It seems plausible that two consecutive zinc finger motifs in a zinc finger protein might be essential for interaction of the protein with DNA...
  67. ncbi request reprint alpha-Synuclein forms a complex with transcription factor Elk-1
    A Iwata
    Laboratory for CAG Repeat Diseases, Molecular Neuropathology Group, RIKEN Brain Science Institute, Wako Shi, Saitama, Japan
    J Neurochem 77:239-52. 2001
    ..Our results suggest that alpha-synuclein reacts with the MAP kinase pathway, which might cause dysfunction of neurons and oligodendrocytes and lead to neurodegeneration in Parkinson's disease and MSA...
  68. ncbi request reprint Characterization and promoter analysis of the mouse gene for transcription factor Sp4
    J Song
    RIKEN The Institute of Physical and Chemical Research, Tsukuba Institute, 3 1 1 Koyadai, Tsukuba, 305 0074, Ibaraki, Japan
    Gene 264:19-27. 2001
    ..We also found that ectopic expression of Sp1 and of MAZ, but not of Sp3, suppressed expression of the Sp4 promoter in a dose-dependent manner...
  69. ncbi request reprint HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain
    M A Kalchman
    Department of Medical Genetics, University of British Columbia, Vancouver, Canada
    Nat Genet 16:44-53. 1997
    ..This provides the first molecular link between huntingtin and the neuronal cytoskeleton and suggests that, in HD, loss of normal huntingtin-HIP1 interaction may contribute to a defect in membrane-cytoskeletal integrity in the brain...
  70. ncbi request reprint Independent repression of a GC-rich housekeeping gene by Sp1 and MAZ involves the same cis-elements
    J Song
    RIKEN (The Insitute of Physical and Chemical Research, Tsukuba Institute, 3-1-1 Koyadai, Tsukuba, Ibaraki 305-0074, Japan
    J Biol Chem 276:19897-904. 2001
    ..Our results indicate that both deacetylation and methylation might be involved in the regulation of expression of a single gene via the actions of different zinc finger proteins that bind to the same cis-elements...
  71. ncbi request reprint Pelizaeus-Merzbacher disease: three novel mutations and implication for locus heterogeneity
    H Osaka
    Department of Pediatrics, School of Medicine, Yokohama City University, Yokohama, Japan
    Ann Neurol 45:59-64. 1999
    ..Moreover, in one family 3 patients exhibited a intragenic polymorphism that did not segregate with the disease, suggesting a locus heterogeneity for Pelizaeus-Merzbacher disease...
  72. ncbi request reprint [On what I learned from researches on Huntington's disease]
    I Kanazawa
    Department of Neurology, School of Medicine, Tokyo University
    Rinsho Shinkeigaku 41:1029-35. 2001
    ..The recruitment of those biologically important proteins into the inclusion bodies could give neurons serious damages for living normally, even if not directly to a catastrophe, a neuronal death...
  73. ncbi request reprint Presence of endothelin-1 in porcine spinal cord: isolation and sequence determination
    O Shinmi
    Department of Biochemistry, University of Tsukuba, Ibaraki, Japan
    Biochem Biophys Res Commun 162:340-6. 1989
    ..The data also indicate that ET-1 may act as a neuropeptide in the central nervous system...
  74. ncbi request reprint A new splicing variant for human tyrosine hydroxylase in the adrenal medulla
    T Ohye
    Institute for Comprehensive Medical Science, Fujita Health University, Toyoake, Aichi 470-1192, Japan
    Neurosci Lett 312:157-60. 2001
    ..The mRNA lacked exon 4, resulting in a premature stop codon at amino acid 147. This result suggests the importance of alternative splicing in the regulation of TH activity...
  75. ncbi request reprint Ataxin-3, the MJD1 gene product, interacts with the two human homologs of yeast DNA repair protein RAD23, HHR23A and HHR23B
    G Wang
    Laboratory for CAG Repeat Diseases, RIKEN Brain Science Institute, 2 1 Hirosawa, Wako Shi, Saitama, 351 0198, Japan
    Hum Mol Genet 9:1795-803. 2000
    ..These results suggest that this interaction is associated with the normal function of ataxin-3 and that some functional abnormality of the HHR23 proteins might exist in MJD...
  76. ncbi request reprint Molecular cloning and expression of two types of rabbit beta-galactoside alpha 1,2-fucosyltransferase
    S Hitoshi
    Molecular Glycobiology, Frontier Research Program, Institute of Physical and Chemical Research RIKEN, Saitama, Japan
    J Biol Chem 270:8844-50. 1995
    ....
  77. ncbi request reprint Evidence for a new variant CYP2D6 allele CYP2D6J in a Japanese population associated with lower in vivo rates of sparteine metabolism
    H Yokota
    Laboratory of Molecular Carcinogenesis, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892
    Pharmacogenetics 3:256-63. 1993
    ..5). In contrast, only two CYP2D6J alleles were found in 14 subjects having MRs of < 1.0. These data suggest that CYP2D6J encodes an enzyme having lower rates of sparteine metabolism...
  78. ncbi request reprint Age-dependent and tissue-specific CAG repeat instability occurs in mouse knock-in for a mutant Huntington's disease gene
    H Ishiguro
    Institute for Comprehensive Medical Science, Fujita Health University, Toyoake Aichi 470 1192, Japan
    J Neurosci Res 65:289-97. 2001
    ..The HD model mouse may therefore have advantages for investigations of molecular mechanisms underlying instability of CAG repeats...
  79. pmc JC virus regulatory region rearrangements in the brain of a long surviving patient with progressive multifocal leukoencephalopathy
    Y Yogo
    Laboratory of Viral Infection, Division of Microbiology and Immunology, The Institute of Medical Science, The University of Tokyo, 4-6-1 Shirokanedai Minato-ku, Tokyo 108-8639, Japan
    J Neurol Neurosurg Psychiatry 71:397-400. 2001
    ..These findings suggested that brain lesions in advanced stages were generated not only by expansion of the original variant (JW-1) of JCV but also by delayed growth of two other variants (JW-3 and 4)...
  80. ncbi request reprint Aberrant processing of the Fugu HD (FrHD) mRNA in mouse cells and in transgenic mice
    K Sathasivam
    Division of Medical and Molecular Genetics, UMDS, Guy s Hospital, London SE1 9RT, UK
    Hum Mol Genet 6:2141-9. 1997
    ..The Fugu HD gene is incorrectly processed in mouse cells both in vitro and in vivo which sheds doubt on the usefulness of Fugu genes for transgenesis...
  81. ncbi request reprint Human huntingtin derived from YAC transgenes compensates for loss of murine huntingtin by rescue of the embryonic lethal phenotype
    J G Hodgson
    Department of Medical Genetics, University of British Columbia, Vancouver, Canada
    Hum Mol Genet 5:1875-85. 1996
    ..These results show that YAC transgenic approaches are a particularly promising route to producing an animal model for disorders associated with CAG expansion...
  82. ncbi request reprint Expression of the alpha1D subunit of the L-type voltage gated calcium channel in human liver
    T Okamoto
    Research Laboratories, Nippon Chemiphar Co, Ltd, 1-22 Hikokawato, Misato, Saitama 341-0005, Japan
    Int J Mol Med 8:413-6. 2001
    ..The alpha1D subunit of the dihydropyridine-sensitive L-type voltage gated calcium channel is expressed relatively strongly in the liver and may play an important role in the liver...
  83. ncbi request reprint Homozygous Machado-Joseph disease presenting as REM sleep behaviour disorder and prominent psychiatric symptoms
    T Fukutake
    Department of Neurology D3, Chiba University Graduate School of Medicine, Chuo Ku, Chiba, Japan
    Eur J Neurol 9:97-100. 2002
    ..In addition to sleep disturbances, in particular RBD, psychiatric symptoms may be important clinical features in both heterozygous and homozygous MJD...