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ホーム  > 神経病理学分野  > Publications

Publications

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2017

Fujita K, Mao Y, Uchida S, Chen X, Shiwaku H, Tamura T, Ito H, Watase K, Homma H, Tagawa K, Sudol M, Okazawa H
Developmental YAPdeltaC determines adult pathology in a model of spinocerebellar ataxia type 1.
Nat Commun, 2017; 8: 1864. doi:10.1038/s41467-017-01790-z.

Okazawa H
Ultra-Early Phase pathologies of Alzheimer's disease and other neurodegenerative diseases.
Proceedings of the Japan Academy. Series B, Physical and biological sciences 93(6) 361-377 2017

Okazawa H
PQBP1, an intrinsically disordered/denatured protein at the crossroad of intellectual disability and neurodegenerative diseases. Neurochemistry international, DOI: 10.1016/j.neuint.2017.06.005.

Sato K, Kerever A, Kamagata K, Tsuruta K, Irie R, Tagawa K, Okazawa H, Arikawa-Hirasawa E, Nitta N, Aoki I, Aoki S
Understanding microstructure of the brain by comparison of neurite orientation dispersion and density imaging (NODDI) with transparent mouse brain. Acta radiologica open 6(4) 2058460117703816


Yamanishi, E., Hasegawa, K., Fujita, K., Ichinose, S., Yagishita, S., Murata, M., Tagawa, K., Akashi, T., Eishi, Y., Okazawa, H. A novel form of necrosis, TRIAD, occurs in human Huntington's disease. Acta Neuropathologica Communications, DOI:10.1186/s40478-017-0420-1

2016

Imamura T, Fujita K, Tagawa K, Ikura T, Chen X, Homma H, Tamura T, Mao Y, Taniguchi JB, Motoki K, Nakabayashi M, Ito N, Yamada K, Tomii K, Okano H, Kaye J, Finkbeiner S, Okazawa H
Identification of hepta-histidine as a candidate drug for Huntington's disease by in silico-in vitro- in vivo-integrated screens of chemical libraries. Scientific reports. 2016 Sep 22;6:33861.

Mao Y, Chen X, Xu M, Fujita K, Motoki K, Sasabe T, Homma H, Murata M, Tagawa K, Tamura T, Kaye J, Finkbeiner S, Blandino G, Sudol M, Okazawa,H..
Targeting TEAD/YAP-transcription-dependent necrosis, TRIAD, ameliorates Huntington's disease pathology. Human molecular genetics. 2016 Sep 12. pii: ddw303.

Fujita K, Motoki K, Tagawa K, Chen X, Hama H, Nakajima K, Homma H, Tamura T, Watanabe H, Katsuno M, Matsumi C, Kajikawa M, Saito T, Saido T, Sobue G, Miyawaki A, Okazawa H..
HMGB1, a pathogenic molecule that induces neurite degeneration via TLR4-MARCKS, is a potential therapeutic target for Alzheimer's disease. Scientific reports. 2016 Aug 25;6:31895.

Taniguchi, JB., Kondo, K., Fujita, K., Chen, X., Homma, H., Sudo, T., Mao, Y., Watase, K., Tanaka, T., Tagawa, K., Tamura, T., Muramatsu, SI., Okazawa, H..
RpA1 ameliorates symptoms of mutant Ataxin-1 knock-in mice and enhances DNA damage repair. Hum. Mol. Genet. 2016.
doi: 10.1093/hmg/ddw272

Mao, Y., Tamura, T., Yuki, Y., Abe, D., Tamada, Y., Imoto, S., Tanaka, H., Homma, H., Tagawa, K., Miyano, S., and Okazawa, H..
The hnRNP-Htt axis regulates necrotic cell death induced by transcriptional repression through impaired RNA splicing. Cell Death & Disease. 2016.04; 7 e2207.

Mizuguchi, M., Obita, T., Kajiyama, A., Kozakai, Y., Nakai, T., Nabeshima, Y. and Okazawa, H. (2016) FEBS Lett. Vol.590 (14): 2221-31. doi:10.1002/1873-3468.12256.

2015

Chen, X., Kondo, k., Motoki, k., Homma, H., Okazawa, H.
Fasting activates macroautophagy in neurons of Alzheimer’s disease mouse model but is insufficient to degrade amyloid-beta. Scientific Reports. 5, Article number: 12115. doi:10.1038/srep12115 Epub date: 14 July 2015

Kamagata, K., Kerever, A., Yokosawa, S., Otake, Y., Ochi, H., Hori, M., Kamiya, K., Tsuruta, K., Tagawa, K., Okazawa, H., Aoki, S., Arikawa-Hirasawa, E.
Quantitative Histological Validation of Diffusion Tensor MRI by Two-Photon Microscopy of Cleared Mouse Brain. Magnetic Resonance in Medical Sciences. Epub date: 30 March 2016

Shiwaku, H., Okazawa, H.
Impaired DNA Damage Repair as a Common Feature of Neurodegenerative Diseases and Psychiatric Disorders. Current Molecular Medicine. 2015; Vol.15 (2) pp119-128. 2015 February
doi: 10.2174/1566524015666150303002556 (Review)

2014

Mizuguchi, M., Obita, T., Serita, T., Kojima, R.,Nabeshima, Y., Okazawa. H.
Mutations in the PQBP1 gene prevent its interaction with the spliceosomal protein U5-15kD. Nature Commun. 5:3822 doi:10.1038/ncomms4822. Pub date: 2014 Apr 30.

Ito, H., Shiwaku, H., Yoshida, C., Homma, H., Luo, H., Chen, X., Fujita, K., Musante, L., Fischer, U., Frint,s SG., Romano, C., Ikeuchi, Y., Shimamura, T., Imoto, S., Miyano, S., Muramatsu, SI., Kawauchi, T., Hoshino, M., Sudol, M., Arumughan, A., Wanker, EE., Rich, T., Schwartz, C., Matsuzaki, F., Bonni, A., Kalscheuer, VM., Okazawa, H.
In utero gene therapy rescues microcephaly caused by Pqbp1-hypofunction in neural stem progenitor cells. Mol Psychiatry. doi: 10.1038/mp.2014.69. Epub date: 2014 July 29.

Nabeshima Y., Mizuguchi M., Kajiyama A., Okazawa H.
Segmental isotope-labeling of the intrinsically disordered protein PQBP1. FEBS Lett. Dec 20;588(24):4583-9. doi: 10.1016/j.febslet.2014.10.028. Epub date: 2014 November 4.

Ito, H., Fujita, K., Tagawa, K., Chen, X., Homma, H., Sasabe, T., Shimizu, J., Shimizu, S., Tamura, T., Muramatsu, S., Okazawa, H.
HMGB1 facilitates repair of mitochondrial DNA damage and extends the lifespan of mutant ataxin-1 knock-in mice. EMBO Mol Med. pii: Dec 15;7(1):78-101. doi: 10.15252/emmm.201404392. Pub date: 2014 December 15.

Shiraishi, R., Tamura, T., Sone, M., Okazawa, H.
Systematic Analysis of Fly Models with Multiple Drivers Reveals Different Effects of Ataxin-1 and Huntingtin in Neuron Subtype-Specific Expression. Dec 31;9(12):e116567. PLoS One. doi: 10.1371/journal.pone.0116567. Epub date: 2014 December 31.

Tagawa, K., Homma, H., Saito, A., Fujita, K., Chen, X., Imoto, S., Oka, T., Ito, H., Motoki, K., Yoshida, C., Hatsuta, H., Murayama, S., Iwatsubo, T., Miyano, S., Okazawa, H.
Comprehensive phosphoproteome analysis unravels the core signaling network that initiates the earliest synapse pathology in preclinical Alzheimer's disease brain. Hum Mol Genet. 2015.01; 24 (2): 540-558. doi: 10.1093/hmg/ddu475 Epub date: 2014 September 17. Pub date: 2015 January 15.

2013

Fujita, K., Nakamura, Y., Oka, T., Ito, H., Tamura, T., Tagawa, K., Sasabe, T., Katsuta, A., Motoki, K., Shiwaku, H., Sone, M., Yoshida, C., Katsuno, M., Eishi, Y., Murata, M., Taylor, JP., Wanker, EE., Kono, K., Tashiro, S., Sobue, G., La, Spada, AR., and Okazawa, H.
A functional deficiency of TERA/VCP/p97 contributes to impaired DNA damage repair in multiple polyglutamine diseases. Nature Commun. 4:1816. doi: 10.1038/ncomms2828

Li, C., Ito, H., Fujita, K., Shiwaku, H., Yunlong Qi, Y., Tagawa, K., Tamura, T., Okazawa, H.
Sox2 transcriptionally regulates Pqbp1, an Intellectual Disability-Microcephaly causative gene, in neural stem progenitor cells. PLOS ONE 8, .e68627. doi: 10.1371/journal.pone.0068627

Shiwaku, H., Yagishita S., Eishi Y., Okazawa, H.
Bergmann glia are reduced in spinocerebellar ataxia type 1. Neuroreport. 24, 620-625. doi: 10.1097/WNR.0b013e32836347b7.

Ikeuchi, Y., de la Torre, L., Matsuda, T., Steen, H., Okazawa, H., Bonni, A.
The XLID protein PQBP1 and the GTPase dynamin 2 define a signaling link that orchestrates ciliary morphogenesis in postmitotic neurons. Cell Reports 4, 1–11. doi:10.1016/j.celrep.2013.07.042

Barclay, S.S., Tamura, T., Ito H., Fujita, K., Tagawa, K., Shimamura, T., Katsuta, A., Shiwaku, H., Sone, M., Imoto, S., Miyano, S. and ○Okazawa, H.
Systems biology analysis of Drosophila in vivo screen data elucidates core networks for DNA damage repair in SCA1. Hum Mol Genet 23:1345-64. doi: 10.1093/hmg/ddt524. Epub 2013 Oct 31.

Mizuguchi, M., Obita, T., Serita, T., Kojima, R., Nabeshima, Y. and Okazawa, H.
Mutations in the PQBP1 gene prevent its interaction with the spliceosomal protein U5-15kD. Nature Commun. 5:3822 doi:10.1038/ncomms4822.

Okazawa, H.
HD Research Around the World: Japan. Past, Present, Future. HD Insights vol. 4, 7-8 (Review)

2012

Ress, M., Gorba, C., Gorba, C., de Chiara, C., Bui, T.T.T., Garcia-Maya, M., Drake, A.F., Okazawa, H., Pastre, A., Svergun, D. and Chen, Y.W.
The solution model of the intrinsically disordered polyglutamine tract binding protein-1 (PQBP-1). Biophys J. 102:1608–1616. doi: 10.1016/j.bpj.2012.02.047

Tamura T., Sone M., Nakamura Y., Shimamura T., Imoto S., Miyano S. and Okazawa H.
A restricted level of PQBP1 is needed for the best longevity of Drosophila. Neurobiology of Aging . 2013 Jan;34(1):356.e11-20. doi: 10.1016/j.neurobiolaging.2012.07.015

2011

Oka, T., Tagawa, K., Ito, H. and Okazawa, H.
Dynamic Changes of the Phosphoproteome in Postmortem Mouse Brains. PLoS One. 6, e21405. doi:10.1371/journal.pone.0021405

Tamura, T., Sone, M., Iwatsubo, T., Tagawa, K., Wanker, E.E. and Okazawa, H.
Ku70 alleviates neurodegeneration in Drosophila models of Huntington's disease. PLoS One. 6, e27408. doi: 10.1371/journal.pone.0027408

Nakamura, Y., Tagawa, K., Oka, T., Sasabe, T., Ito, H., Shiwaku, H., La Spada, A.R. and Okazawa, H.
Ataxin-7 associates with microtubules and stabilizes the cytoskeletal network. Hum Mol Genet. 21 (5): 1099-1110. doi: 10.1093/hmg/ddr539

Ress, M., Gorba, C., Gorba, C., de Chiara, C., Bui, T.T.T., Garcia-Maya, M., Drake, A.F., Okazawa, H., Pastre, A., Svergun, D. and Chen, Y.W.
The solution model of the intrinsically disordered polyglutamine tract binding protein-1 (PQBP-1). Biophys J. 102:1608–1616. doi: 10.1016/j.bpj.2012.02.047


Enokido, Y., Okazawa, H.
DNA Repair in the Nervous System: A New Research for Neurological Disorders. in DNA Repair: New Research edited by Kimura, S. and Shimizu, S. Nova Science. (ISBN: 978-1-62100-756-2)