Hosoi N, Shibasaki K, Hosono M, Konno A, Shinoda Y, Kiyonari H, Inoue K, Muramatsu SI, Ishizaki Y, Hirai H, Furuichi T, Sadakata T
Deletion of class II ARFs in mice causes tremor by the Nav1.6 loss in cerebellar Purkinje cell axon initial segments.
J Neurosci. 2019 Jun 14. pii: 2002-18. [Epub ahead of print]

Sato M, Seki T, Konno A, Hirai H, Kurauchi Y, Hisatsune A, Katsuki H
Rapamycin activates mammalian microautophagy.
J Pharmacol Sci. 2019 May 24. pii: S1347-8613(19)34062-9. [Epub ahead of print]

Matsuzaki Y, Tanaka M, Hakoda S, Masuda T, Miyata R, Konno A, Hirai H
Neurotropic Properties of AAV-PHP.B Are Shared among Diverse Inbred Strains of Mice
Mol Ther. 2019 Apr 10;27(4):700-704. Epub 2019 Feb 28.

Cendelin J, Buffo A, Hirai H, Magrassi L, Mitoma H, Sherrard R, Vozeh F, Manto M
Task Force Paper On Cerebellar Transplantation: Are We Ready to Treat Cerebellar Disorders with Cell Therapy?
Cerebellum. 2019 Jan 3.   [Epub ahead of print]

Watanave M, Hoshino C, Konno A, Fukuzaki Y, Matsuzaki Y, Ishitani T, Hirai H
Pharmacological enhancement of retinoid-related orphan receptor α function mitigates spinocerebellar ataxia type 3 pathology.
Neurobiol Dis. 2019 Jan;121:263-273. Epub 2018 Oct 19.

Clinical Neuroscience. 2019 Vol.37 (19年) 08月号: 998-1002

今野歩, 平井宏和
実験医学. 2019; 37(4): 581-587



Watanave M, Matsuzaki Y, Nakajima Y, Ozawa A, Yamada M, Hirai H
Contribution of Thyrotropin-Releasing Hormone to Cerebellar Long-Term Depression and Motor Learning.
Front Cell Neurosci. 2018 Dec 12.

Seki T, Sato M, Konno A, Hirai H, Kurauchi Y, Hisatsune A, Katsuki H.
d-Cysteine promotes dendritic development in primary cultured cerebellar Purkinje cells via hydrogen sulfide production.
Mol Cell Neurosci. 2018 Dec;93:36-47. Epub 2018 Oct 19.

Shinohara Y, Konno A, Nitta K, Matsuzaki Y, Yasui H, Suwa J, Hiromura K, Hirai H.
Effects of Neutralizing Antibody Production on AAV-PHP.B-Mediated Transduction of the Mouse Central Nervous System.
Mol Neurobiol. 2019 Jun;56(6):4203-4214. Epub 2018 Oct 5.

Seki T, Sato M, Kibe Y, Ohta T, Oshima M, Konno A, Hirai H, Kurauchi Y, Hisatsune A, Katsuki H.
Lysosomal dysfunction and early glial activation are involved in the pathogenesis of spinocerebellar ataxia type 21 caused by mutant transmembrane protein 240.
Neurobiol Dis. 2018 Sep 2. pii: S0969-9961(18)30519-9.

Gaffield MA, Rowan MJM, Amat SB, Hirai H, Christie JM.
Inhibition gates supralinear Ca2+ signaling in Purkinje cell dendrites during practiced movements.
Elife. 2018 Aug 17;7. pii: e36246.

Hirai H and Kano M.
Type 1 metabotropic glutamate receptor and its signaling molecules as therapeutic targets for the treatment of cerebellar disorders.
Curr Opin Pharmacol. 2018 Feb;38:51-58.

Hirai H.
Protein Kinase C in the Cerebellum: Its Significance and Remaining Conundrums.
Cerebellum. 2018 Feb;17(1):23-27.

Ogawa Y, Kakumoto K, Yoshida T, Kuwako KI, Miyazaki T, Yamaguchi J, Konno A, Hata J, Uchiyama Y, Hirai H, Watanabe M, Darnell RB, Okano H, Okano HJ
Elavl3 is essential for the maintenance of Purkinje neuron axons.
Sci Rep. 2018 Feb 9;8(1):2722.

Matsuzaki Y, Konno A, Mochizuki R, Shinohara Y, Nitta K, Okada Y, Hirai H
Intravenous administration of the adeno-associated virus-PHP.B capsid fails to upregulate transduction efficiency in the marmoset brain.
Neurosci Lett. 2018 Feb 5;665:182-188. Epub 2017 Nov 24.

脳科学辞典. 2018. doi:10.14931/bsd.7623

脳科学辞典. 2018. doi:10.14931/bsd.7632



Takahashi N, Shuvaev AN, Konno A, Matsuzaki Y, Watanave M, Hirai H.
Regulatory connection between the expression level of classical protein kinase C and pruning of climbing fibers from cerebellar Purkinje cells.
J Neurochem. 2017 Dec;143(6):660-670. Epub 2017 Nov 29.

Nitta K, Matsuzaki Y, Konno A, Hirai H
Minimal Purkinje Cell-Specific PCP2/L7 Promoter Virally Available for Rodents and Non-human Primates.
Mol Ther Methods Clin Dev. 2017 Jul 27;6:159-170.

Harada K, Ito M, Wang X, Tanaka M, Wongso D, Konno A, Hirai H, Hirase H, Tsuboi T, Kitaguchi T.
Red fluorescent protein-based cAMP indicator applicable to optogenetics and in vivo imaging.
Scientific Reports. 2017 Aug 4;7(1):7351

Hattori T, Kaji M, Ishii H, Jureepon R, Takarada-Iemata M, Minh Ta H, Manh Le T, Konno A, Hirai H, Shiraishi Y, Ozaki N, Yamamoto Y, Okamoto H, Yokoyama S, Higashida H, Kitao Y, Hori O.
CD38 positively regulates postnatal development of astrocytes cell-autonomously and oligodendrocytes non-cell-autonomously.
Glia. 2017 Jun;65(6):974-989. Epub 2017 Mar 13.

Shinohara Y, Ohtani T, Konno A, Hirai H
Viral Vector-Based Evaluation of Regulatory Regions in the Neuron-Specific Enolase (NSE) Promoter in Mouse Cerebellum In Vivo.
Cerebellum. 2017 Dec;16(5-6):913-922.

Matsuzaki Y, Konno A, Mukai R, Honda F, Hirato M, Yoshimoto Y, Hirai H.
Transduction Profile of the Marmoset Central Nervous System Using Adeno-Associated Virus Serotype 9 Vectors.
Molecular Neurobiology. 2017 Apr;54(3):1745-1758. Epub 2016 Feb 16.

Gonçalves N, Simões AT, Prediger RD, Hirai H, Cunha RA, Pereira de Almeida L
Caffeine alleviates progressive motor deficits in a transgenic mouse model of spinocerebellar ataxia.
Ann Neurol. 2017 Mar;81(3):407-418. Epub 2017 Feb 17.

Shuvaev AN, Hosoi N, Sato Y, Yanagihara D, Hirai H
Progressive impairment of cerebellar mGluR signalling and its therapeutic potential for cerebellar ataxia in spinocerebellar ataxia type 1 model mice.
Journal of Physiology. 2017 Jan 1;595(1):141-164. Epub 2016 Sep 15.

全国脊髄小脳変性症(SCD)・多系統萎縮症(MSA)友の会ニュース. No. 226: 10-14. 2017.04.25発行

生化学. 2017; 89(2): 241-243. doi:10.14952/SEIKAGAKU.2017.890241

Clinical Neuroscience. 35巻01月号: 28-32. 2017.01.01発行



Arsenault J, Gholizadeh S, Niibori Y, Pacey LK, Halder SK, Koxhioni E, Konno A, Hirai H, Hampson DR.
FMRP Expression Levels in Mouse CNS Neurons Determine Behavioral Phenotype.
Human Gene Therapy. 2016 Dec;27(12):982-996. Epub 2016 Sep 7.

Ito E, Tomaru Y, Iizuka A, Hirai H, Kato T.
Adaptive Local Thresholding for Co-Localization Detection in Multi-Channel Fluorescence Microscopic Images.
IEICE Transactions on Information and Systems. 2016 Nov 1;99(11):2851-2855.

Huda F, Fan Y, Suzuki M, Konno A, Matsuzaki Y, Takahashi N, Chan JK, Hirai H.
Fusion of Human Fetal Mesenchymal Stem Cells with “Degenerating” Cerebellar Neurons in Spinocerebellar Ataxia Type 1 Model Mice.
PLoS One. 2016 Nov 1;11(11):e0164202.

Shinohara Y, Konno A, Takahashi N, Matsuzaki Y, Kishi S, Hirai H.
Viral Vector-Based Dissection of Marmoset GFAP Promoter in Mouse and Marmoset Brains.
PLoS One. 2016 Aug 29;11(8):e0162023.

Takahashi H, Ogawa Y, Yoshihara S, Asahina R, Kinoshita M, Kitano T, Kitsuki M, Tatsumi K, Okuda M, Tatsumi K, Wanaka A, Hirai H, Stern PL, Tsuboi A.
A Subtype of Olfactory Bulb Interneurons Is Required for Odor Detection and Discrimination Behaviors.
Journal of Neuroscience. 2016 Aug 3;36(31):8210-27.

Sato M, Seki T, Konno A, Hirai H, Kurauchi Y, Hisatsune A, Katsuki H.
Fluorescent-based evaluation of chaperone-mediated autophagy and microautophagy activities in cultured cells.
Genes Cells. 2016 Aug;21(8):861-73. Epub 2016 Jul 5.

Sawada Y, Konno A, Nagaoka J, Hirai H.
Inflammation-induced reversible switch of the neuron-specific enolase promoter from Purkinje neurons to Bergmann glia.
Scientific Reports. 2016 Jun 13;6:27758.

Yanai T, Kurosawa A, Nikaido Y, Nakajima N, Saito T, Osada H, Konno A, Hirai H, Takeda S.
Identification and molecular docking studies for novel inverse agonists of SREB, super conserved receptor expressed in brain.
Genes Cells. 2016 Jul;21(7):717-27. Epub 2016 May 17.

Suto N, Mieda T, Iizuka A, Nakamura K, Hirai H.
Morphological and Functional Attenuation of Degeneration of Peripheral Neurons by Mesenchymal Stem Cell-Conditioned Medium in Spinocerebellar Ataxia Type 1-Knock-in Mice.
CNS Neuroscience & Therapeutics. 2016 Aug;22(8):670-6. Epub 2016 May 3.

Monai H, Ohkura M, Tanaka M, Oe Y, Konno A, Hirai H, Mikoshiba K, Itohara S, Nakai J, Iwai Y, Hirase H.
Calcium imaging reveals glial involvement in transcranial direct current stimulation-induced plasticity in mouse brain.
Nature Communications. 2016 Mar 22;7:11100.

Conceição M, Mendonça L, Nóbrega C, Gomes C, Costa P, Hirai H, Moreira JN, Lima MC, Manjunath N, Pereira de Almeida L.
Intravenous administration of brain-targeted stable nucleic acid lipid particles alleviates Machado-Joseph disease neurological phenotype.
Biomaterials. Volume 82, March 2016, Pages 124–137

Conceição M, Mendonça L, Nóbrega C, Gomes C, Costa P, Hirai H, Moreira JN, Lima MC, Manjunath N, Pereira de Almeida L.
Safety profile of the intravenous administration of brain-targeted stable nucleic acid lipid particles.
Data Brief. 2016 Jan 20;6:700-5.



Mieda T, Suto N, Iizuka A, Matsuura S, Iizuka H, Takagishi K, Nakamura K, Hirai H.
Mesenchymal stem cells attenuate peripheral neuronal degeneration in spinocerebellar ataxia type 1 knockin mice.
J Neurosci Res. 2016 Mar;94(3):246-52. Epub 2015 Dec 28.

Ageta-Ishihara N, Yamazaki M, Konno K, Nakayama H, Abe M, Hashimoto K, Nishioka T, Kaibuchi K, Hattori S, Miyakawa T, Tanaka K, Huda F, Hirai H, Hashimoto K, Watanabe M, Sakimura K, Kinoshita M.
A CDC42EP4/septin-based perisynaptic glial scaffold facilitates glutamate clearance.
Nat Commun. 2015 Dec 10;6:10090.

Nóbrega C, Carmo-Silva S, Albuquerque D, Vasconcelos-Ferreira A, Vijayakumar UG, Mendonça L, Hirai H, Pereira de Almeida L.
Re-establishing ataxin-2 downregulates translation of mutant ataxin-3 and alleviates Machado-Joseph disease.
Brain 2015 Dec;138(Pt 12):3537-54.

Duarte-Neves J, Gonçalves N, Cunha-Santos J, Simões AT, den Dunnen WF, Hirai H, Kügler S, Cavadas C, Pereira de Almeida L.
Neuropeptide Y mitigates neuropathology and motor deficits in mouse models of Machado-Joseph disease.
Hum Mol Genet 2015 Oct 1;24(19):5451-63.

Iizuka A, Matsuzaki Y, Konno A, Hirai H.
Plasticity of the developmentally arrested staggerer cerebellum in response to exogenous RORα.
Brain Structure and Function 2016 Jul;221(6):2879-89. Epub 2015 Jun 30.

Kusakari S, Saitow F, Ago Y, Shibasaki K, Sato-Hashimoto M, Matsuzaki Y, Kotani T, Murata Y, Hirai H, Matsuda T, Suzuki H, Matozaki T, Ohnishi H.
Shp2 in forebrain neurons regulates synaptic plasticity, locomotion, and memory formation in mice.
Molecular and Cellular Biology 2015 May 1;35(9):1557-72.

Iizuka A, Nakamura K, Hirai H.
Long-term oral administration of the NMDA receptor antagonist memantine extends life span in spinocerebellar ataxia type 1 knock-in mice.
Neurosci Lett 2015 Apr 10;592:37-41.

Nakamura K, Mieda T, Suto N, Matsuura S, Hirai H.
Mesenchymal Stem Cells as a Potential Therapeutic Tool for Spinocerebellar Ataxia.
Cerebellum 2015 Apr;14(2):165-70.

Mendonça LS, Nóbrega C, Hirai H, Kaspar BK, Pereira de Almeida L
Transplantation of cerebellar neural stem cells improves motor coordination and neuropathology in Machado-Joseph disease mice.
Brain 2015 Feb;138(Pt 2):320-35.

Uesaka N, Uchigashima M, Mikuni T, Hirai H, Watanabe M, Kano M.
Retrograde Signaling for Climbing Fiber Synapse Elimination.
Cerebellum 2015 Feb;14(1):4-7.

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 2015 Jan 15;24(2):525-39.

脊髄小脳変性症・多系統萎縮症 Q&A 172 平成27年3月発行



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.

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.



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.


「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


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



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.


News & Hot Paper Digest
羊土社・実験医学 Vol.30 No.1, 64-65, 2012 Jan



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]


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.

News & Hot Paper Digest
羊土社・実験医学 Vol.29 No.13, 2130, 2011 Aug

News & Hot Paper Digest
羊土社・実験医学 Vol.29 No.6, 904-905, 2011 Apr



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, Hira H.
High Transgene Expression by Lentiviral Vectors Causes Maldevelopment of Purkinje Cells in Vivo.
The Cerebellum 2010 Sep;9(3):291-302.


News & Hot Paper Digest
羊土社・実験医学 Vol.28 No.9, 1401-1402, 2010 June



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 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.


平井 宏和
【特集 遺伝子-脳回路-行動】
生体の科学. Vol.60 No.1, 23-30, 2009

News&Hot Paper Digest
羊土社・実験医学. Vol.3, 104-405, 2009

V. 生殖医学 A. 基礎分野での進歩
Annual Review 糖尿病・代謝・内分泌. 中外医学社, 196-201, 2009



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.


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
難病と在宅ケア Vol.14 No.2(5月号), 43-46, 2008



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.


平井 宏和、東田陽博
羊土社・実験医学. Vol.25 No.11(7月号) 1707-1710, 2007

平井 宏和
クバプロ・ブレインサイエンス レビュー2007, 123-140



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
羊土社・実験医学 Vol.24 No.19(12月号) 2006, 2997-3001

平井 宏和
羊土社・実験医学 Vol.24 No.15(増刊) 2006, 192(2402)