Irie T, Matsuzaki Y, Sekino Y, Hirai H.
Kv3.3 channels harboring a mutation of spinocerebellar ataxia type 13 alterexcitability and induce cell death in cultured cerebellar Purkinje cells.
The Journal of Physiology 2014 Jan 1;592(Pt 1):229-47.

Konno A, Shuvaev AN, Miyake N, Miyake K, Iizuka A, Matsuura S, Huda F, Nakamura K, Yanagi S, Shimada T, Hirai H.
Mutant Ataxin-3 with an Abnormally Expanded Polyglutamine Chain Disrupts Dendritic Development and Metabotropic Glutamate Receptor Signaling in Mouse Cerebellar Purkinje Cells.
The Cerebellum 2014 Feb;13(1):29-41.

Matsuzaki Y, Oue M, Hirai H.
Generation of a neurodegenerative disease mouse model using lentiviral vectors carrying an enhanced synapsin I promoter.
The Journal of Neuroscience Methods 2014 Feb 15;223:133-43.


Matsuura S, Shuvaev AN, Iizuka A, Nakamura K, Hirai H.
Mesenchymal Stem Cells Ameliorate Cerebellar Pathology in a Mouse Model of Spinocerebellar Ataxia Type 1.
The Cerebellum 2014 Jun;13(3):323-30.

Okonogi N, Nakamura K, Suzuki Y. Suto N. Suzue K. Kaminama T. Nakano T. Hirai H.
Cranial irradiation induces bone marrow-derived microglia in adult mouse brain tissue.
Journal of Radiation Research 2014 Jul;55(4):713-9.


Uesaka N, Uchigashima M, Mikuni T, Nakazawa T, Nakao H, Hirai H, Aiba A, Watanabe M, Kano M.
Retrograde semaphorin signaling regulates synapse elimination in the developing mouse brain.
Science 2014 May 30;344(6187):1020-3.

Huda F, Konno A. Matsuzaki Y. Goenawan H. Miyake K. Shimada T and Hirai H.
Distinct transduction profiles in the CNS via three injection routes of AAV9 and the application to generation of a neurodegenerative mouse model.
Molecular Therapy-Methods & Clinical Development 2014 Aug 6.

Saida H, Matsuzaki Y, Takayama K, Iizuka A, Konno A, Yanagi S, Hirai H.
One-year follow-up of transgene expression by integrase-defective lentiviral vectors and their therapeutic potential in spinocerebellar ataxia model mice.
Gene Therapy 2014 Sep;21(9):820-7.

Takahashi H, Adachi N, Shirafuji T, Danno S, Ueyama T, Vendruscolo M, Shuvaev AN, Sugimoto T, Seki T, Hamada D, Irie K, Hirai H, Sakai N, Saito N.
Identification and characterization of PKCγ, a kinase associated with SCA14, as an amyloidogenic protein.
Human Molecular Genetics 2014 Sep 12.

Yoshihara S, Takahashi H, Nishimura N, Kinoshita M, Asahina R, Kitsuki M. Tatsumi K, Furukawa-Hibi Y, Hirai H, Nagai T, Yamada K, Tsuboi A.
Npas4 regulates Mdm2 and thus Dcx in experience-dependent dendritic spine development of newborn olfactory bulb interneurons.
Cell Reports 2014 Aug 7;8(3):843-57.

Nakamura K, Mieda T, Suto N, Matsuura S, Hirai H.
Mesenchymal Stem Cells as a Potential Therapeutic Tool for Spinocerebellar Ataxia.
Cerebellum 2014 Oct 4. [Epub ahead of print]

Uesaka N, Uchigashima M, Mikuni T, Hirai H, Watanabe M, Kano M.
Retrograde Signaling for Climbing Fiber Synapse Elimination.
Cerebellum 2014 Oct 23. [Epub ahead of print]



Nóbrega C, Nascimento-Ferreira I, Onofre I, Albuquerque D, Hirai H, Déglon N, de Almeida LP.
Silencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice.
PLoS One
2013;8(1):e52396.

Takechi Y, Mieda T, Iizuka A, Toya S, Suto N, Takagishi K, Nakazato Y, Nakamura K, Hirai H.
Impairment of spinal motor neurons in spinocerebellar ataxia type 1-knock-in mice.

Neuroscience Letters
2013 Feb 22;535:67-72.

Liu HX, Lopatina O, Higashida C, Fujimoto H, Akther S, Inzhutova A, Liang M, Zhong J, Tsuji T, Yoshihara T, Sumi K, Ishiyama M, Ma WJ, Ozaki M, Yagitani S, Yokoyama S, Mukaida N, Sakurai T, Hori O, Yoshioka K, Hirao A, Kato Y, Ishihara K, Kato I, Okamoto H, Cherepanov SM, Salmina AB, Hirai H, Asano M, Brown DA, Nagano I, Higashida H
Displays of paternal mouse pup retrieval following communicative interaction with maternal mates.
Nature Communications
2013;4:1346.


Yamaura H. Hirai H, Yanagihara D.
Postural dysfunction in a transgenic mouse model of spinocerebellar ataxia type 3.
Neuroscience 2013 Jul 23;243:126-35.

Mikuni T, Uesaka N, Okuno H, Hirai H, Deisseroth K, Bito H, Kano M.
Arc/Arg3.1 is a postsynaptic mediator of activity-dependent synapse elimination in the developing cerebellum.
Neuron 2013 Jun 19;78(6):1024-35.

Nascimento-Ferreira I, Nóbrega C, Vasconcelos-Ferreira A, Onofre I, Albuquerque D, Aveleira C, Hirai H, Déglon N, Pereira de Almeida L.
Beclin 1 mitigates motor and neuropathological deficits in genetic mouse models of Machado-Joseph disease.
Brain 2013 Jul;136(Pt 7):2173-88.

Akther S, Korshnova N, Zhong J, Liang M, Cherepanov SM, Lopatina O, Komleva YK, Salmina AB, Nishimura T, Fakhrul AA, Hirai H, Kato I, Yamamoto Y, Takasawa S, Okamoto H, Higashida H.
CD38 in the nucleus accumbens and oxytocin are related to paternal behavior in mice.
Molecular Brain 2013 Sep 23;6(1):41.

Miki T, Hirai H, Takahashi T.
Activity-dependent neurotrophin signaling underlies developmental switch of Ca2+ channel subtypes mediating neurotransmitter release.
The Journal of Neuroscience 2013 Nov 27;33(48):18755-63.



Jin D, Muramatsu SI, Shimizu N, Yokoyama S, Hirai H, Yamada K, Liu HX, Higashida C, Hashii M, Higashida A, Asano M, Ohkuma S, Higashida H.

Dopamine release via the vacuolar ATPase V0 sector c-subunit, confirmed in N18 neuroblastoma cells, results in behavioral recovery in hemiparkinsonian mice.

Neurochemistry International
2012 Nov;61(6):907-12

Yoshihara SI, Takahashi H, Nishimura N, Naritsuka H, Shirao T, Hirai H, Yoshihara Y, Mori K, Stern PL, Tsuboi A.
5T4 Glycoprotein Regulates the SensoryInput-Dependent Development of a Specific Subtype of Newborn Interneurons inthe Mouse Olfactory Bulb.
Journal of Neuroscience 2012 Feb 8;32(6):2217-2226.

Hirai H.
Basic research on cerebellar gene therapy using lentiviral vectors.
The Cerebellum
2012 Jun 11(2): 443-5

Uesaka N. Mikuni T. Hashimoto K. Hirai H. Sakimura K. Kano M.
Organotypic Coculture Preparation for the Study of Developmental SynapseElimination in Mammalian Brain.
Journal of Neuroscience
2012 Aug 22; 32(34): 11657-11670

Goenawan H. Hirai H.
Modulation of lentiviral vector tropism in cerebellar Purkinje cells in vivo by a lysosomal cysteine protease cathepsin K.
Journal of NeuroVirology
2012 Dec;18(6):521-31

Oue M, Handa H, Matsuzaki Y, Suzue K, Murakami H, Hirai H.
The murine stem cell virus promoter drives correlated transgene expression in the leukocytes and cerebellar Purkinje cells of transgenic mice.
PLoS One 2012;7(11):e51015.



Mitsumura K, Hosoi N, Furuya N, Hirai H.
Disruption of metabotropic glutamate receptor signalling is a major defect at cerebellar parallel fibre-Purkinje cell synapses in staggerer mutant mice.
The Journal of Physiology 2011 May 9; 589:3191-3209. Epub 2011 Jul 1

Shuvaev AN, Horiuchi H, Seki T, Goenawan H, Irie T, Iizuka A, Sakai N, Hirai H.
Mutant PKC{gamma} in spinocerebellar ataxia type 14 disrupts synapse elimination and long-term depression in Purkinje cells in vivo.
The Journal of Neuroscience 2011 Oct 5;31(40):14324-34.

Hirai H.
Basic research on cerebellar gene therapy using lentiviral vectors.
The Cerebellum
2011 Nov 26. [Epub ahead of print]



Sasaki J, Kofuji S, Itoh R, Momiyama T, Takayama K, Murakami H, Chida S, Tsuya Y, Takasuga S, Eguchi S, Asanuma K, Horie Y, Miura K, Davies EM, Mitchell C, Yamazaki M, Hirai H, Takenawa T, Suzuki A, Sasaki T.
The PtdIns(3,4)P(2) phosphatase INPP4A is a suppressor of excitotoxic neuronal death.
Nature
2010 May 27;465(7297):497-501. Epub 2010 May 12.

Munesue T, Yokoyama S, Nakamura K, Anitha A, Yamada K, Hayashi K, Asaka T, Liu HX, Jin D, Koizumi K, Islam MS, Huang JJ, Ma WJ, Kim UH, Kim SJ, Park K, Kim D, Kikuchi M, Ono Y, Nakatani H, Suda S, Miyachi T, Hirai H, Salmina A, Pichugina YA, Soumarokov AA, Takei N, Mori N, Tsujii M, Sugiyama T, Yagi K, Yamagishi M, Sasaki T, Yamasue H, Kato N, Hashimoto R, Taniike M, Hayashi Y, Hamada J, Suzuki S, Ooi A, Noda M, Kamiyama Y, Kido MA, Lopatina O, Hashii M, Amina S, Malavasi F, Huang EJ, Zhang J, Shimizu N, Yoshikawa T, Matsushima A, Minabe Y, Higashida H.
Two genetic variants of CD38 in subjects with autism spectrum disorder and controls.
Neuroscience Research
2010 Jun;67(2):181-91. Epub 2010 May 1

Sawada Y, Kajiwara G, Iizuka A, Takayama K, Shuvaev AN, Koyama C, Hirai H.
High Transgene Expression by Lentiviral Vectors Causes Maldevelopment of Purkinje Cells in Vivo.
The Cerebellum 2010 Sep;9(3):291-302.



Iizuka A, Takayama K, Torashima T, Yamasaki M, Koyama C, Mitsumura K, Watanabe M, Hirai H.
Lentiviral vector-mediated rescue of motor behavior in spontaneously occurring hereditary ataxic mice.
Neurobiology of Disease 2009 Sep;35(3):457-65. Epub 2009 Jun 30.

Torashima T, Iizuka A, Horiuchi H, Mitsumura K, Yamasaki M, Koyama C, Takayama K, IinoM, Watanabe M, Hirai H.
Rescue of abnormal phenotypes in delta2 glutamate receptor-deficient mice by the extracellular N-terminal and intracellular C-terminal domains of the delta2 glutamate receptor.

European Journal of Neuroscience 2009 Aug;30(3):355-65. Epub 2009 Jul 15

Oue M, Mitsumura K, Torashima T, Koyama C, Yamaguchi H, Furuya N, Hirai H. 
Characterization of mutant mice that express polyglutamine in cerebellar Purkinje cells.
Brain Research
2009 Feb 19;1255:9-17.

Shiba K, Torashima T, Hirai H, Ogawa K, Akhter N, Nakajima K, Kinuya S, Mori H.
Potential usefulness of D(2)R reporter gene imaging by IBF as gene therapy monitoring for cerebellar neurodegenerative diseases.
Journal of Cerebral Blood Flow & Metabolism 2009 Feb;29(2):434-40.

Kobayashi T, Hirai H, Iino M, Fuse I, Mitsumura K, Washiyama K, Kasai S, Ikeda K.
Inhibitory effects of the antiepileptic drug ethosuximide on G protein-activated inwardly rectifying K+ channels.
Neuropharmacology 2009 Feb;56(2):499-506.



Sato T, Torashima T, Sugihara K, Hirai H, Asano M, Yoshioka K.
The scaffold protein JSAP1 regulatesproliferation and differentiation of cerebellar granule cell precursors bymodulating JNK signaling.
Molecular and Cellular Neuroscience 2008 Dec;39(4):569-78. Epub 2008 Aug 30.

Takayama K, Torashima T, Horiuchi H, Hirai H
Purkinje-cell-preferential transduction by lentiviral vectors with the murine stem cell virus promoter.
Neuroscience Letters 2008 Sep 26;443(1):7-11. Epub 2008 Jul 25.

Hirai H.
Progress in transduction of cerebellar Purkinje cells in vivo using viral vectors.
The Cerebellum
2008 7(3):273-8.

Han L, Suda M, Tsuzuki K, Wang R, Ohe Y, Hirai H, Watanabe T, Takeuchi T, Hosaka M.
A large form of secretogranin III functions as a sorting receptor for chromogranin A aggregates in PC12 cells.
Molecular Endocrinology
2008 Aug;22(8):1935-49. Epub 2008 May 15.

Torashima T, Koyama C, Iizuka A, Mitsumura K, Takayama K, Yanagi S, Oue M, Yamaguchi H, Hirai H.
Lentivector-mediated rescue from cerebellar ataxia in a mouse model of spinocerebellar ataxia.
EMBO Reports
2008 Apr;9(4):393-9. Epub 2008 Mar 14.



Torashima T, Koyama C, Higashida H, Hirai H.
Production of neuron-preferential lentiviral vectors.
Nature Protocols
Epub 2007 Feb 14.

Nakamura K, Hirai H, Torashima T, Miyazaki T, Tsurui H, Xin Y, Ohtsuji M, Shun Lin Q, Tsukamoto K, Nishimura H, Ono M, Watanabe M and Hirose S.
CD3 and immunoglobulin G fc receptor regulate cerebellar functions.
Molecular and Cell Biology
2007 Jul;27(14):5128-34, 2007.

Jin D*, Liu HX*, Hirai H*, Torashima T, Nagai T, Lopatina O, Shnayder NA, Yamada K, Noda M, Seike T, Fujita K, Takasawa S, Yokoyama S, Koizumi K, Shiraishi Y, Tanaka S, Hashii M, Yoshihara T, Higashida K, Islam MS, Yamada N, Hayashi K, Noguchi N, Kato I, Okamoto H, Matsushima A, Salmina A, Munesue T, Shimizu N, Mochida S, Asano M and Higashida H. (*equally contributed)
CD38 is critical for social behaviour by regulating oxytocin secretion.
Nature
2007 Mar 1;446(7131):41-5, 2007.

Kakegawa W, Miyazaki T, Hirai H, Motohashi J, Mishima M, Watanabe M, Yuzaki M.
Ca2+ permeability of the channel pore is not essential
for the {delta}2 glutamate receptor to regulate synaptic plasticity
and motor coordination.

Journal of Physiology 2007 Mar 15;579(Pt 3):729-35, 2007.



Hisatsune C, Kuroda Y, Akagi T, Torashima T, Hirai H, Hashikawa T, Inoue T, Mikoshiba K
Inositol 1, 4, 5-Trisphosphate Receptor Type 1 in Granule Cells, not in Purkinje Cells, Regulates the Dendritic Morphology of Purkinje Cells through BDNF Production.
J. Neurosci. 18;26(42):10916-10924, 2006

Torashima T, Yamada N, Itoh M, Yamamoto A, Hirai H.
Exposure of lentiviral vectors to subneutral pH shifts the tropism from Purkinje cell to Bergmann glia.
Eur .J. Neurosci
. Jul 24:371-380, 2006

Torashima T, Okoyama S, Nishizaki T, Hirai H.
In vivo transduction of murine cerebellar Purkinje cells by HIV-derived lentiviral vectors.
Brain Res
. Apr 12;1082(1):11-22, 2006

Qin Q, Inatome R, Hotta A, Kojima M, Yamamura H, Hirai H, Yoshizawa T, Tanaka H, Fukami K, and Yanagi S.
A novel GTPase, CRAG, mediates PML-associated nuclear body formation and degradation of
expanded polyglutamine protein. 
J. Cell. Biol. 172:497-504, 2006

Takaturu Y., Takayasu Y., Iino M., Nikkuni O., Ueda Y., Tanaka K.,and Ozawa S.
Roles of glial glutamate transporters in shaping EPSCs at the climbing fiber-Purkinje cell synapses.
Neurosci. Res. 54:140-148, 2006

Takayasu Y., Iino M., Shimamoto K., Tanaka K. and Ozawa S.
Glial glutamate transporters maintain One-to-One relationship at the limbing fiber-Purkinje cell synapse by preventing glutamate spillover
J. Neurosci. 26: 6563-6572., 2006

Tsuzuki K, Tricoire L, Gibelin N, Bourout G, Rossier J, Lambolez B.  
Calcium dependence of aequorin bioluminescence dissected by random mutagenesis.
Proc. Natl. Acad. Sci. U.S.A  103: 9500-9505, 2006



平井宏和
「第5章 小脳への遺伝子導入」
小脳と運動失調 小脳はなにをしているのか(アクチュアル 脳・神経疾患の臨床)

(ISBN: 978-4-521-73442-2) 辻省次(編集), 西澤正豊(編集) 中山書店


平井宏和

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「神経変性疾患でなぜ神経細胞が死ぬのか?」
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Hirai H, Iizuka A.
Chapter 22. Recent developments in gene therapy research targeted to cerebellar disorders.
Gene Therapy Applications
(ISBN:978-953-307-541-9). Edited by Chunsheng Kang, Publisher: InTech, Aug 2011.


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「脳のマクロファージ、ミクログリアはどこからやってくるのか?」

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平井 宏和
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羊土社・実験医学
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平井宏和(著書、分担執筆)
V. 生殖医学 A. 基礎分野での進歩
「最近の知見からみるオキシトシンと母性の新しい概念」
Annual Review 糖尿病・代謝・内分泌.
中外医学社, 196-201, 2009



平井 宏和
脊髄小脳変性症 SCDマウスの運動障害を遺伝子治療で改善
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平井 宏和、東田陽博
「子育て」「他者の認識と記憶」に必要な膜タンパク質CD38

羊土社・実験医学.
 Vol.25 No.11(7月号) 1707-1710, 2007


平井 宏和
グルタミン酸受容体リン酸化と運動学習制御
クバプロ・ブレインサイエンス レビュー2007, 123-140

平井 宏和
神経細胞選択的なHIV由来レンチウィルスベクターの作製法
羊土社・実験医学 Vol.24 No.19(12月号) 2006, 2997-3001

平井 宏和
小脳シナプス形成とシナプス可塑性を制御する分子・細胞メカニズム
羊土社・実験医学 Vol.24 No.15(増刊) 2006, 192(2402)



Hirokazu Hirai
Progress of spinocerebellar ataxia research on pathology, model animals and gene/stem cell therapies: Toward clinical application
第19回日本遺伝子治療学会学術集会・シンポジウム(S2-2) 2013年7月5日

Hirokazu Hirai
Virus-mediated highly efficient and cell-type specific gene expression in the cerebellum - Modulation of cathepsin K activity and development of unique promoters -
第90回日本生理学会大会・シンポジウム(1S13E-1) 2013年3月27日