ಽሶ␹⚻↢‛ቇಽ㊁ Σ ⎇ⓥ⚵❱㧔᭴ᚑຬ ᐔᚑᐕ᦬ᣣ⃻࿷㧕   ᢎ᝼㧦    ㇊ᵄብਯ     ᛛⴚ⡯ຬ㧦    ⨹ᧁ৻᣿   ಎᢎ᝼㧦    ᱞ੗ᑧਯ     ඳ჻⺖⒟ᄢቇ㒮↢㧦ဈ੗⟤๺ሶ   ഥᢎ㧦     㔍ᵄኼ᣿              ḡᎹዅⴕ   ഥᢎ㧦     ጤୖ⊖วሶฎᎹ๺㇢
․છഥᢎᄖጊ⧷๺Τ ⎇ⓥᵴേ
㧝㧕⛔วᄬ⺞∝ߩ∛࿃∛ᘒ⸃᣿ߩὑ‫ޔ‬േ‛ࡕ࠺࡞ߩ㐿⊒⸃ᨆ߿ㆮવሶ⸃ᨆࠍⴕߞߡ޿ࠆ‫ޕ‬')(ࡕ࠺࡞േ‛ࠍ㐿⊒ߒ‫ⴕޔ޿↪ࠍ࠻࠶࠮ࡕ࡯ࡑޔ࠻࠶࡜ޔࠬ࠙ࡑޔ‬േ‫⚦ޔ‬⢩‫ޔ‬ಽሶ࡟ࡌ࡞ߢ
ߩ⸃ᨆࠍⴕߞߡ޿ࠆ‫ޕ‬㧞㧕')(ࡈࠔࡒ࡝࡯ಽሶߣ'TD$Uߩ࡝ࠟࡦ࠼г ฃኈ૕⋧੕૞↪‫࡯࡝ࡒࠔࡈ()'ޔ‬ಽሶߩࠪࠚ࠺ࠖࡦࠣ
ᯏ᭴ߩ⹦⚦ߥᬌ⸛‫ޔ‬෸߮␹⚻⚦⢩ߦኻߔࠆ૞↪ߦߟ޿ߡ⸃ᨆࠍⴕߞߡ޿ࠆ‫ޕ‬㧟㧕㔤㐳㘃ߩࠫࡦࠢࡈࠖࡦࠟ࡯ㆮવሶߦ⌕⋡ߒ‫ޔ‬ฦ㔤㐳㘃ߩᲧセࠥࡁࡓ߆ࠄ‫ޔ‬ㅴൻߦࠃࠆᯏ⢻⊛ᄌൻࠍᬌ⸛ߒߡ޿ࠆ‫ޕ‬㧠㧕␹⚻⚦⢩ߦ߅ߌࠆ⠡⸶ᯏ᭴ߩࡔࠞ࠾࠭ࡓࠍ⸃ᨆߒߡ޿ࠆ‫ޕ‬㧡㧕O614ࠪࠣ࠽࡞♽ߩ⸃ᨆࠍⴕߞߡ޿ࠆ‫ޕ‬Υ ⺰ᢥ㧔ේ⪺‫଀∝ޔ⺑✚ޔ‬ႎ๔ࠍ඙೎ߒߥ޿㧕1) Takei N, Furukawa K, Hanyu O, Sone H, Nawa H. A possible link between BDNF andmTOR in control of food intake. Front Psychol. 2014 Sep 25;5:1093. doi:
10.3389/fpsyg.2014.01093. eCollection 2014.
2) Nawa H, Sotoyama H, Iwakura Y, Takei N, Namba H. Neuropathologic implication of
peripheral neuregulin-1 and EGF signals in dopaminergic dysfunction and behavioral deficits
relevant to schizophrenia: their target cells and time window. Biomed Res Int.
2014;2014:697935. doi: 10.1155/2014/697935.
3) Takei N, Nawa H. mTOR signaling and its roles in normal and abnormal brain development.
Front Mol Neurosci. 2014 Apr 23;7:28. doi: 10.3389/fnmol.2014.00028. eCollection 2014.
4) Kunii Y, Miura I, Matsumoto J, Hino M, Wada A, Niwa S, Nawa H, Sakai M, Someya T,
Takahashi H, Kakita A, Yabe H.Elevated postmortem striatal t-DARPP expression inschizophrenia
andassociations
with
DRD2/ANKK1
polymorphism.
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Neuropsychopharmacol
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4;53:123-8.
doi:
10.1016/j.pnpbp.2014.03.014.
5) Bundo M, Toyoshima M, Okada Y, Akamatsu W, Ueda J, Nemoto-Miyauchi T, Sunaga F,
Toritsuka M, Ikawa D, Kakita A, Kato M, Kasai K, Kishimoto T, Nawa H, Okano H,
Yoshikawa T, Kato T, Iwamoto K. Increased l1 retrotransposition in the neuronal genome inschizophrenia. Neuron. 2014 Jan 22;81(2):306-13. doi: 10.1016/j.neuron.2013.10.053
㸫㸫Φ ౒ห⎇ⓥ   ⎇ⓥ㗴⋡㧦‫⛔ޟ‬วᄬ⺞∝⣖ౝߢߩ૕⚦⢩ㆮવሶᄌ⇣‫ޠ‬   ⎇ⓥౝኈ㧦⣖ߩࠥࡁࡓ⸃ᨆߦࠃࠅ‫⛔ޔ‬วᄬ⺞∝␹⚻⚦⢩ߦ߅ߌࠆ૕⚦⢩ᄌ⇣ࠍត⚝ߔࠆ‫ޕ‬   ෳടᯏ㑐㧦ℂൻቇ⎇ⓥᚲ ℂൻቇ⎇ⓥᚲ⣖⑼ቇ✚ว⎇ⓥ࠮ࡦ࠲࡯ ട⮮ᔘผ  ⎇ⓥ㗴⋡㧦‫․ࠍ࠻ࡅޟ‬ᓽߠߌࠆ⣖Ყセ࠻࡜ࡦࠬࠢ࡝ࡊ࠻࡯ࡓ࡮Ყセࡔ࠴ࡠ࡯ࡓ⸃ᨆ‫ޠ‬ ⎇ⓥౝኈ㧦ࡅ࠻෸߮㔤㐳㘃ߢࠫࡦࠢࡈࠖࡦࠟ࡯ㆮવሶ⟲ߦ⌕⋡ߒ‫⃻⊒ޔ‬Ყセ෸߮ࠥࡁࡓࡔ࠴࡞ൻᲧセࠍⴕ޿‫․ࠍ࠻ࡅޔ‬ᓽߠߌࠆㆮવሶࠍត⚝ߔࠆ‫ޕ‬   ෳടᯏ㑐㧦੩ㇺᄢቇ㔤㐳㘃⎇ⓥᚲ ㇹ ᐽᐢ   ⎇ⓥ㗴⋡㧦‫⛔ޟ‬วᄬ⺞∝ߦ߅ߌࠆ࠼ࡄࡒࡦࠪࠣ࠽࡞ߩᄌ⺞‫ޠ‬   ⎇ⓥౝኈ㧦ᱫᓟ⣖ࠍ↪޿ߡ⛔วᄬ⺞∝ߦ߅ߌࠆ࠼ࡄࡒࡦ㑐ㅪಽሶߩࠥࡁࡓ⸃ᨆ෸߮⊒⃻⸃ᨆࠍⴕ߁‫ޕ‬   ෳടᯏ㑐㧦⑔ፉ⋵┙ක⑼ᄢቇ ࿡੗ᵏੱ   ⎇ⓥ㗴⋡㧦‫ޟ‬ᖚ⠪ K25 ⚦⢩߆ࠄߩ࠼ࡄࡒࡦ␹⚻ಽൻ⢻ജߩᲧセ‫ޠ‬   ⎇ⓥౝኈ㧦⛔วᄬ⺞∝ᖚ⠪߆ࠄ᮸┙ߒߚ K25 ⚦⢩ߩ࠼ࡄࡒࡦ␹⚻⚦⢩߳ߩಽൻ⢻ജࠍᲧセߔࠆ‫ޕ‬   ෳടᯏ㑐㧦ℂൻቇ⎇ⓥᚲ ℂൻቇ⎇ⓥᚲ⣖⑼ቇ✚ว⎇ⓥ࠮ࡦ࠲࡯ ศᎹᱞ↵  ⎇ⓥ㗴⋡㧦‫ޟ‬㔤㐳㘃ࠍ߽ߜ޿ߚ⛔วᄬ⺞∝ࡕ࠺࡞േ‛ߩ૞ᚑ‫ޠ‬   ⎇ⓥౝኈ㧦ࡑ࡯ࡕ࠮࠶࠻ࠍ↪޿ߚ⛔วᄬ⺞∝ࡕ࠺࡞ߩ᮸┙ࠍ⋡ᜰߔ‫ޕ‬   ෳടᯏ㑐㧦੩ㇺᄢቇ㔤㐳㘃⎇ⓥᚲ ਛ᧛స᮸
㸫㸫⚦⢩␹⚻↢‛ቇಽ㊁Σ ⎇ⓥ⚵❱㧔᭴ᚑຬ ᐔᚑᐕ᦬ᣣ⃻࿷㧕   ᢎ᝼    㦮᧛ ᑪม     ታ㛎⵬ഥ     ᄢၳ ජᵗ   ಎᢎ᝼   㒙ㇱ ቇ      ታ㛎⵬ഥ     ᦸ᦬ 㔐⛗   ഥᢎ    ਛോ ⢩      ታ㛎⵬ഥ     ੗Ỉ ⡡ሶ   ഥᢎ    ౝ↰ ੳม     ታ㛎⵬ഥ     ⇟႐ ᓀሶ․છഥᢎ   ๟  㤀      ታ㛎⵬ഥ     ⍹ᧄ ⩿Ⓞሶ   ᛛⴚ⡯ຬ  ᰏ⼱ ᖝሶ     ⒁ᦠ       ㊁Ỉ ૫਎ᛛⴚ⡯ຬ  ᄐ⋡ ㉿ᕺᄢቇ㒮↢㧔ඳ჻㧕 㐳Ỉ ኼ⏴⎇ⓥຬ   Ꮉ᧛ ฬሶ     ᄢቇ㒮↢㧔ඳ჻㧕 ᓄ  ⪊⎇ⓥຬ   ㋈ᧁ ື਎     ᄢቇ㒮↢㧔ඳ჻㧕 ㋈ᧁ ᐽᶈ⎇ⓥຬ   ᷰㄝ ๺ᴰ     ᄢቇ㒮↢㧔ඳ჻㧕 ਛᧄ ජ዆ታ㛎⵬ഥ  ⍫ㇱ ᕺ⒩ሶ    ᄢቇ㒮↢㧔ୃ჻㧕 ⷏Ꮉ ජ᥏ታ㛎⵬ഥ  ⍹Ꮉ ⵨೑ሶΤ ⎇ⓥᵴേ
 ᧄಽ㊁ߢߪ⣖㜞ᰴᯏ⢻ߩಽሶᯏ᭴⸃᣿ࠍ⋡⊛ߣߒߡ‫ࠬࡊ࠽ࠪޔ‬વ㆐‫ޔ‬น႟ᕈߦ㊀ⷐߥᓎഀࠍᨐߚߔ
ಽሶࠍᮡ⊛ߣߒߚㆮવሶᡷᄌࡑ࠙ࠬࠍ%$.0⚐♽⢦ᕈᐙ⚦⢩ࠍ↪޿ߡ૞⵾ߒ‫ߩߘޔ‬ಽሶߩ↢ℂᯏ⢻ࠍ⸃ᨆߒߡ޿ࠆ‫ޕ‬ᒰ⹥ᐕᐲߢߪ‫⑼ޔ‬ቇ⎇ⓥ⾌⵬ഥ㊄‫ࠆߌ߅ߦࠬࡊ࠽ࠪޟ‬ㅒⴕᕈࠪࠣ࠽࡞ߦࠃࠆᯏ⢻⊛␹⚻࿁〝ᒻᚑߩᯏ᭴⸃᣿‫ޟޔޠ‬㜞ᰴ⣖㗔ၞߦ߅ߌࠆࠪ࠽ࡊࠬવ㆐೙ᓮᯏ᭴ߩಽሶᒻᘒቇ⊛⎇ⓥ‫ࠍ╬ޠ‬ฃߌߡ⎇ⓥࠍㆀⴕߒ‫㉄ࡦࡒ࠲࡞ࠣޔ‬ฃኈ૕ߩ↢ℂᯏ⢻߿‫ޔ‬ౝ࿃ᕈࠞࡦ࠽ࡆࡁࠗ࠼ߦࠃࠆࠪ࠽ࡊࠬવ㆐೙
ᓮᯏ᭴ߩ৻┵ࠍ᣿ࠄ߆ߦߔࠆߥߤ‫ޔ‬ਥߣߒߡࠪ࠽ࡊࠬવ㆐ߦ㑐ࠊࠆቇⴚ⺰ᢥᧄߩ⊒⴫ߦ⽸₂ߒߚ‫ޕ‬߹ߚ‫ޟ‬ᣂẟᄢቇ⣖⎇ⓥᚲ౒ห೑↪࡮౒ห⎇ⓥ‫ߡߒߣޠ‬ઙߩ౒ห⎇ⓥࠍⴕ޿‫ޟޔ‬൮᜝ဳ⣖⑼ቇ⎇ⓥផ
ㅴᡰេࡀ࠶࠻ࡢ࡯ࠢ ࡝࠰࡯ࠬ࡮ᛛⴚᡰេ‫ߡߒߣޠ‬ઙߩࡑ࠙ࠬ૞⵾ᡰេࠍⴕߞߚ‫⑼ޔߦࠄߐޕ‬ቇ⎇ⓥ⾌⵬ഥ㊄‫࠻ࡅޟ‬⣖ᯏ⢻⸃᣿߳ߩ㆏⒟ߣߒߡߩㆮવሶᡷᄌ࡜࠶࠻૞⵾ᴺߩ㐿⊒‫ࠍޠ‬ฃߌ‫↢ޔ‬ᱺવ㆐♽೉น⢻ߥ࡜࠶࠻⢦ᕈᐙ⚦⢩ߩ㐿⊒ࠍⴕߞߚ‫ޕ‬Υ ⺰ᢥ㧔ේ⪺‫଀∝ޔ⺑✚ޔ‬ႎ๔ࠍ඙೎ߒߥ޿㧕1.

Kitajima Y, Tashiro Y, Suzuki N, Warita H, Kato M, Tateyama M, Ando R, Izumi R, Yamazaki M, Abe M, Sakimura K, Ito
H, Urushitani M, Nagatomi R, Takahashi R, Aoki M.㩷 䋺㩷 Proteasome dysfunction induces muscle growth defects andprotein aggregation.㩷 J Cell Sci. 2014 Dec 15;127(24):5204-17. doi: 10.1242/jcs.150961.

2.

Yasumura M, Yoshida T, Yamazaki M, Abe M, Natsume R, Kanno K, Uemura T, Takao K, Sakimura K, Kikusui T,
Miyakawa T, Mishina M.㩷 䋺㩷 IL1RAPL1 knockout mice show spine density decrease, learning deficiency, hyperactivity
and reduced anxiety-like behaviours.㩷 Sci Rep. 2014 Oct 14;4:6613. doi: 10.1038/srep06613.

3.

Otsu Y, Marcaggi P, Feltz A, Isope P, Kollo M, Nusser Z, Mathieu B, Kano M, Tsujita M, Sakimura K, Dieudonné S.㩷 䋺㩷
Activity-Dependent Gating of Calcium Spikes by A-type K+ Channels Controls Climbing Fiber Signaling in Purkinje Cell
Dendrites.㩷 Neuron. 2014 Oct 1;84(1):137-51. doi: 10.1016/j.neuron.2014.08.035.

㸫㸫

4.

Kaneko R, Abe M, Hirabayashi T, Uchimura A, Sakimura K, Yanagawa Y, Yagi T.㩷 䋺㩷 Expansion of stochastic
expression repertoire by tandem duplication in mouse Protocadherin-Į cluster.㩷 Sci Rep. 2014 Sep 2;4:6263. doi:
10.1038/srep06263.

5.

Yamasaki M, Okada R, Takasaki C, Toki S, Fukaya M, Natsume R, Sakimura K, Mishina M, Shirakawa T, Watanabe
M.䋺㩷 Opposing Role of NMDA Receptor GluN2B and GluN2D in Somatosensory Development and Maturation.㩷 J
Neurosci. 2014 Aug 27;34(35):11534-48. doi: 10.1523/JNEUROSCI.1811-14.2014.

6.

Kawata S, Miyazaki T, Yamazaki M, Mikuni T, Yamasaki M, Hashimoto K, Watanabe M, Sakimura K, Kano M. : Global
Scaling Down of Excitatory Postsynaptic Responses in Cerebellar Purkinje Cells Impairs Developmental Synapse
Elimination. Cell Rep. 2014 Aug 21;8(4):1119-29. doi: 10.1016/j.celrep.2014.07.014.

7.

Itoi K, Talukder AH, Fuse T, Kaneko T, Ozawa R, Sato T, Sugaya T, Uchida K, Yamazaki M, Abe M, Natsume R,
Sakimura K.: Visualization of Corticotropin-Releasing Factor Neurons by Fluorescent Proteins in the Mouse Brain andCharacterization of Labeled Neurons in the Paraventricular Nucleus of the Hypothalamus. Endocrinology. 2014
Oct;155(10):4054-60. doi: 10.1210/en.2014-1182.

8.

Harayama T, Eto M, Shindou H, Kita Y, Otsubo E, Hishikawa D, Ishii S, Sakimura K, Mishina M, Shimizu T.㩷 䋺
Lysophospholipid Acyltransferases Mediate Phosphatidylcholine Diversification to Achieve the Physical Properties
Required In Vivo.㩷 Cell Metab. 2014 Aug 5;20(2):295-305. doi: 10.1016/j.cmet.2014.05.019.

9.

Petrenko AB, Yamazaki M, Sakimura K, Kano M, Baba H.㩷 䋺Augmented tonic pain-related behavior in knockout micelacking monoacylglycerol lipase, a major degrading enzyme for the endocannabinoid 2-arachidonoylglycerol.㩷 Behav
Brain Res. 2014 Sep 1;271:51-8. doi: 10.1016/j.bbr.2014.05.063..

10. Konno K, Matsuda K, Nakamoto C, Uchigashima M, Miyazaki T, Yamasaki M, Sakimura K, Yuzaki M, Watanabe M. :Enriched Expression of GluD1 in Higher Brain Regions and Its Involvement in Parallel Fiber-Interneuron Synapse
Formation in the Cerebellum. J Neurosci. 2014 May 28;34(22):7412-24.doi: 10.1523/JNEUROSCI.0628-14.2014.
11. Hartmann J, Karl RM, Alexander RP, Adelsberger H, Brill MS, Rühlmann C, Ansel A, Sakimura K, Baba Y, Kurosaki T,
Misgeld T, Konnerth A : STIM1 Controls Neuronal Ca(2+) Signaling, mGluR1-Dependent Synaptic Transmission, andCerebellar Motor Behavior. Neuron. 2014 May 7;82(3):635-44. doi: 10.1016/j.neuron.2014.03.027.
12. He X, Ishizeki M, Mita N, Wada S, Araki Y, Ogura H, Abe M, Yamazaki M, Sakimura K, Mikoshiba K, Inoue T, Ohshima
T. : Cdk5/p35 is required for motor coordination and cerebellar plasticity. J Neurochem.㩷 2014 Oct;131(1):53-64. doi:
10.1111/jnc.12756.
13. Ikegami M, Uemura T, Kishioka A, Sakimura K, Mishina M.㩷 䋺㩷 Striatal dopamine D1 receptor is essential forcontextual fear conditioning.㩷 Sci Rep. 2014 Feb 5;4:3976. doi: 10.1038/srep03976.
14. Horváth E, Woodhams SG, Nyilas R, Henstridge CM, Kano M, Sakimura K, Watanabe M, Katona I.㩷 䋺㩷 Heterogeneouspresynaptic distribution of monoacylglycerol lipase, a multipotent regulator of nociceptive circuits in the mouse spinal
cord.㩷 Eur J Neurosci. 㩷 2014 Feb;39(3):419-34. doi: 10.1111/ejn.12470.
15. Fujita H, Aoki H, Ajioka I, Yamazaki M, Abe M, Oh-Nishi A, Sakimura K, Sugihara I.㩷 䋺㩷 Detailed expression pattern ofaldolase C (aldoc) in the cerebellum, retina and other areas of the CNS studied in aldoc-venus knock-in mice.㩷 PLoS
One. 2014 Jan 27;9(1):e86679. doi: 10.1371/journal.pone.0086679. eCollection 2014.
㸫㸫Φ ౒ห⎇ⓥ   ⎇ⓥ㗴⋡  ‫ޟ‬ᣂẟᄢቇ⣖⎇ⓥᚲ ౒ห೑↪࡮౒ห⎇ⓥ‫ޠ‬   ⎇ⓥౝኈ   %$.♽⛔'5⚦⢩ࠍ↪޿ߚㆮવሶᡷᄌࡑ࠙ࠬߩ૞⵾ᡰេ   ෳടᯏ㑐  ⥄ὼ⑼ቇ⎇ⓥᯏ᭴‫᧲ޔ‬ർᄢቇ‫᧲ޔ‬੩ᄢቇ‫ޔ‬㑐⷏ක⑼ᄢቇ   ⎇ⓥ㗴⋡  ‫ޟ‬൮᜝ဳ⣖⑼ቇ⎇ⓥផㅴᡰេࡀ࠶࠻ࡢ࡯ࠢ‫ޠ‬   ⎇ⓥౝኈ   ⁛ഃᕈߩ㜞޿⣖ࡕ࠺࡞േ‛‫ࠬ࠙ࡑ࡞࠺ࡕޔ‬⣖ᯏ⢻⸃ᨆߦ․ൻߒߚㆮવሶᡷᄌ
ࡑ࠙ࠬ૞⵾ᡰេ   ෳടᯏ㑐  ⥄ὼ⑼ቇ⎇ⓥᯏ᭴‫ޔ‬੩ㇺᄢቇ‫᧲ޔ‬੩ᄢቇ‫ޔ‬ᣂẟᄢቇ߶߆   ⎇ⓥ㗴⋡  ‫ࠆߌ߅ߦࠬࡊ࠽ࠪޟ‬ㅒⴕᕈࠪࠣ࠽࡞ߦࠃࠆᯏ⢻⊛␹⚻࿁〝ᒻᚑߩᯏ᭴⸃᣿‫ޠ‬   ⎇ⓥౝኈ   ౝ࿃ᕈࠞࡦ࠽ࡆࡁࠗ࠼วᚑ෸߮ಽ⸃ߦ㑐ਈߔࠆಽሶ⟲ߩㆮવሶᡷᄌࡑ࠙ࠬ૞
  ⵾‫⸃ޔ‬ᨆ‫ޔ‬ଏਈ ෳടᯏ㑐    ᧲੩ᄢቇ‫ޔ‬ᓼፉᢥℂᄢቇ‫ޔ‬ᣂẟᄢቇ   ⎇ⓥ㗴⋡  ‫ޟ‬㜞ᰴ⣖㗔ၞߦ߅ߌࠆࠪ࠽ࡊࠬવ㆐೙ᓮᯏ᭴ߩಽሶᒻᘒቇ⊛⎇ⓥ‫ޠ‬   ⎇ⓥౝኈ   ࠪ࠽ࡊࠬવ㆐೙ᓮߦ㑐ਈߔࠆಽሶ⟲ߩㆮવሶᡷᄌࡑ࠙ࠬ૞⵾‫⸃ޔ‬ᨆ‫ޔ‬ଏਈ ෳടᯏ㑐    ർᶏ㆏ᄢቇ‫ޔ‬ᣂẟᄢቇ 㧔㧕⎇ⓥ㗴⋡  ‫ޟ‬%$.♽⛔↱᧪'5⚦⢩ࠍ↪޿ߚฦ⒳ㆮવሶᡷᄌേ‛ߩ⸳⸘ߣ૞ᚑ‫ޠ‬⎇ⓥౝኈ   ࠪ࠽ࡊࠬᒻᚑߦ㑐ਈߔࠆಽሶ⟲ߩㆮવሶᡷᄌࡑ࠙ࠬߩ૞⵾‫⸃ޔ‬ᨆ‫ޔ‬ଏਈෳടᯏ㑐  ᘮᙥᄢቇ‫ޔ‬ർᶏ㆏ᄢቇ‫ޔ‬ᣂẟᄢቇ㧔㧕⎇ⓥ㗴⋡  ‫ޟ‬ㆮવሶᡷᄌേ‛ߩ૞⵾ߦ᦭↪ߥ㧱㧿⚦⢩ߩ૞ᚑ࡮⹏ଔ‫ޠ‬⎇ⓥౝኈ  %$.↱᧪'5⚦⢩4'0-#ࠍ↪޿ߚ‫ޔ‬ㆮવሶᡷᄌࡑ࠙ࠬ૞⵾ᣇᴺߦ㑐ߔࠆᣂⷙ
ᛛⴚ㐿⊒ෳടᯏ㑐  ᩣᑼળ␠࠻࡜ࡦࠬࠫࠚ࠾࠶ࠢ‫ޔ‬ᣂẟᄢቇ㸫㸫ࢩࢫࢸ࣒⬻⏕⌮Ꮫศ㔝 Ϩ ◊✲⤌⧊㸦ᵓᡂဨ ᖹᡂᖺ᭶᪥⌧ᅾ㸧   ᩍᤵ⃅ᮌ ඞᰤ   ෸ᩍᤵ ⳻⏣ ❳୍ຓᩍሯ㔝 ᾈ᫂ຓᩍྜྷṊ ㅮᖹ

≉ู◊✲ဨ㙊㇂ ኱ᶞᢏ⾡⫋ဨ୸ᒣ బⱥᏊᢏ⾡⫋ဨᯇᓥ ⥤஀

༤ኈㄢ⛬኱Ꮫ㝔⏕㛫ᗞ ᆂ୍㸦ᩚᙧእ⛉㸧ᒣᓊ 㐩ஓ㸦⪥㰯⛉㸧኱す Ẏ 㸦㯞㓉⛉㸧ϩ ◊✲άື
ᡃࠎࡣࠊ⤒㢌⵹ࣇࣛࣅࣥ⺮ⓑ⺯ග࢖࣓࣮ࢪࣥࢢࢆ୺せ࡞◊✲ᡭẁ࡜ࡋ࡚ࠊ࣐࢘ࢫ኱⬻⓶㉁ឤぬ㔝㸦⫈ぬ㔝࣭どぬ㔝࣭యᛶឤぬ㔝㸧ࠊཬࡧ௦⾲ⓗ࡞ឤぬ㐃ྜ㔝࡜ࡋ࡚どぬ⣔㐃ྜ㔝ࡢᶵ⬟ࢆゎᯒࡋ࡚࠸ࡿࠋどぬ⣔㐃ྜ㔝ࡣࠊ✵㛫᝟ሗࢆྲྀࡾᢅ࠺⫼ഃ⤒㊰࡜ᙧ≧᝟ሗࢆྲྀࡾᢅ࠺⭡ഃ⤒㊰࡟኱ูࡉࢀࡿࠋ㟋㛗㢮࡛▱ࡽࢀࡓࡇࡢᵓ㐀ࡣࠊᇶᮏⓗ࡟ࡣ࣐࢘ࢫ࡟ࡶᏑᅾࡋࠊ⫼ഃ⤒㊰ࡣᚋ㒊㢌㡬㐃ྜ㔝ࢆ⤊╔Ⅼ࡜ࡍࡿࠋᚋ㒊㢌㡬㐃ྜ㔝ࡢᶵ⬟࡜ࡋ࡚ࡣࠊ✵㛫᝟ሗࡢ▷ᮇグ᠈ࡸどぬ᝟ሗ࡜యᛶឤぬ᝟ሗࡢ⤫ྜ࡞࡝ࠊࡑࡢാࡁࡀᚎࠎ࡟ุࡗ࡚ࡁࡓࠋࡋ࠿ࡋࠊ࣐࢘ࢫࡢ⭡ഃ⤒㊰ࡀ࡝ࡢ⛬ᗘⓎ㐩ࡋ࡚࠸ࡿࡢ࠿ࠊ⤊╔Ⅼࡢᙧ≧ㄆ▱ࡢ㈐௵㒊఩ࡣ࡝ࡇ࡟Ꮡᅾࡍࡿࡢ࠿ࠊヲࡋࡃุࡗ࡚࠸࡞࠸ࠋ௒ᖺᗘࡢ◊✲࡜ࡋ࡚࣐࢘ࢫ࡟࠾ࡅࡿ⭡ഃ⤒㊰ࡢ⤊╔Ⅼࠊ༶ࡕᙧ≧ㄆ▱ࡢ㈐௵㒊఩࡟㛵ࡋ࡚▱ぢࢆᚓࡘࡘ࠶ࡿ
ࡢ࡛⤂௓ࡋࡓ࠸ࠋ
ᡃࠎࡣ࣐࢘ࢫࡢᙧ≧ㄆ▱⬟ຊࢆホ౯ࡍࡿࡓࡵࠊ㹋Ꮠ㏞㊰ἲࢆ㛤Ⓨࡋࡓࠋࡇࢀࡣ㹋Ꮠᆺࡢ㏞㊰ࡢୖ⦕㒊ศ࡟ᾮᬗ⏬㠃ࢆタ⨨ࡋࠊࡑࡇ࡟ᥦ♧ࡋࡓ⏬ീ᝟ሗ࡟ᇶ࡙࠸࡚㏞㊰ࢆ㑅ᢥࡉࡏࡿ⿦⨨࡛࠶ࡿࠋࡇࡢ㹋Ꮠ㏞㊰ࢆ⏝࠸ࡿ࡜ࠊ࣐࢘ࢫࡣᑡ࡞ࡃ࡜ࡶ㸰㸮⛊㛫ࡣࡸ࡞࡝ࡢ」㞧࡞ᅗᙧࢆグ᠈ࡋࠊಖᣢࡍࡿ⬟ຊࡀ࠶ࡿࡇ࡜ࡀุࡗࡓࠋ㦫࠸ࡓࡇ࡜࡟ࠊࡇࡢࡼ࠺࡟カ⦎ࡉࢀࡓ࣐࢘ࢫࡣࠊ⏕ࡲࢀ࡚ึࡵ࡚ぢࡓ࢔ࣝࣇ࢓࣋ࢵࢺࡢᙧ≧ࢆグ᠈ࡋࠊࡸ࡞࡝࡜ྠᵝ࡟㸰㸮⛊㛫ಖᣢ࡛ࡁࡿࡇ࡜ࡀุࡗࡓࠋ
༶ࡕ࣐࢘ࢫࡣࠊ」㞧࡞ᅗᙧ࡟ྵࡲࢀࡿከࡃࡢ᝟ሗ㔞ࢆ⣲᪩ࡃグ᠈࣭ಖᣢࡍࡿ㧗ᗘ࡞どぬグ᠈⬟ຊࢆ᭷ࡋ࡚࠸ࡿࠋ
ࡑࢀ࡛ࡣ࣐࢘ࢫࡢᙧ≧ㄆ▱ࡢ㈐௵㒊఩ࡣ࡝ࡢࡼ࠺࡟ྠᐃ࡛ࡁࡿ࡛࠶ࢁ࠺࠿㸽」㞧࡞ᅗᙧࢆᥦ♧ࡋ࡚どぬ㔝ࢆάືࡉࡏࡿ࡜ࠊᅗᙧ࡟㑅ᢥⓗ࡞ᛂ⟅ࡀฟࡿࡔࡅ࡛࡞ࡃࠊ᫂ᬯࡸᅗᙧࢆᵓᡂࡍࡿ⥺ศ࡟ᛂࡎࡿ㠀㑅ᢥⓗ࡞ᛂ⟅ࡶฟ⌧ࡋࠊᅗᙧ㑅ᢥⓗᛂ⟅࡜㠀㑅ᢥⓗᛂ⟅ࢆ༊ูࡍࡿࡇ࡜ࡣᅔ㞴࡛࠶ࡿࠋࡋ࠿ࡋࠊ౛࠼ࡤணࡵ」㞧࡞ᅗᙧ࡜」㞧࡞㡢ࢆ㐃᝿௜ࡅࠊ㡢่⃭࡟ࡼࡗ࡚ᅗᙧࢆ㐃᝿ࡉࡏࡓሙྜࡣࠊ
᫂ᬯࡸᅗᙧࢆᵓᡂࡍࡿ⥺ศ࡟ᑐࡍࡿ㠀㑅ᢥⓗ࡞ᛂ⟅ࢆ㉳ࡇࡍࡇ࡜࡞ࡃࠊ┤᥋ᅗᙧ㑅ᢥⓗ࡞ᛂ⟅ࢆ㉳ࡇࡍࡇ࡜ࡀ࡛ࡁࡿࡣࡎ࡛࠶ࡿࠋ
ࡑࡇ࡛ᡃࠎࡣࡲࡎ࣐࢘ࢫࡢ㐃᝿グ᠈⬟ຊࢆࢸࢫࢺࡋࡓࠋ࡜㡢$ࠊ࡜㡢%ࢆ㐃᝿௜ࡅࡿ࡜ࠊ࣐
࢘ࢫࡣ㡢࠿ࡽࡸࢆ㐃᝿ࡋࠊ㐃᝿ࡋࡓᅗᙧ࡜ྠࡌࡶࡢࢆ㑅ᢥࡍࡿ⬟ຊࡀ࠶ࡿࡇ࡜ࢆࠊ㹋Ꮠ㏞㊰ἲ࡛☜ㄆࡋࡓࠋࡉࡽ࡟ࡇࡢࡼ࠺࡞࣐࢘ࢫࡢ㡢࡟ᑐࡍࡿ⓶㉁ᛂ⟅ࡀࠊୖࡢᶍᘧᅗ࡟♧ࡋࡓࡼ࠺࡟⫈㸫㸫ぬ㔝ࡢ⫼ഃ㸦どぬ㔝ࡢ⭡ഃ㸧࡟ฟ⌧ࡋࡓࠋࡇࡢ㒊ศࡢᛂ⟅ࢆගᏊ࢖࣓࣮ࢪࣥࢢ࡛ゎᯒࡍࡿ࡜ࠊ
ࡸ࡞࡝࡟ᛂࡎࡿࢽ࣮ࣗࣟࣥࡢ௚࡟ࠊ㐃᝿௜ࡅ࡟⏝࠸ࡓ㡢࡟ᛂࡎࡿࢽ࣮ࣗࣟࣥࡶグ㘓ࡉࢀࡓࠋࡋ
࠿ࡶ㐃᝿௜ࡅ࡟⏝࠸ࡓ㡢࡜ᅗᙧ࡟ᛂࡎࡿࢽ࣮ࣗࣟࣥࡣ㞄ࡾྜࡗ࡚Ꮡᅾࡍࡿሙྜࡀ࠶ࡗࡓࠋࡇࡢࡼ
࠺࡟㞄᥋ࡍࡿࢽ࣮ࣗࣟࣥ࣌࢔ࡀ㐃᝿௜ࡅ࡟⏝࠸ࡿࡑࢀࡒࢀࡢ่⃭࡟ᛂࡌࠊࡉࡽ࡟ࢽ࣮ࣗࣟࣥ࣌࢔
㛫ࡢ⤖ྜࡀᙉ໬ࡉࢀࡿࡇ࡜ࡀ㐃᝿グ᠈ࡢ࣓࢝ࢽࢬ࣒࡛࠶ࡿྍ⬟ᛶࡀ࠶ࡿࠋ
ࡉࡽ࡟᫖ᖺ࡟ᘬࡁ⥆ࡁF3FGK㑇ఏᏊࡢከᵝᛶࡢᶵ⬟ࢆ᳨ドࡍࡿࡓࡵࠊ✀㢮ࡢF3FGKDࢡࣛࢫࢱ࣮
ࡢ࠺ࡕࠊD࠿ࡽDࡲ࡛ࡀḞᦆࡋࠊD㸯࠿D㸯㸰ࡢ࡝ࡕࡽ࠿ࡀⓎ⌧ࡍࡿ࣐࢘ࢫ㸦F3FGKD࣐࢘ࢫ㸧ࡢゎᯒࢆ⾜ࡗࡓࠋࡲࡎ㹋Ꮠ㏞㊰ࢆ⏝࠸ࡓ◊✲࡛ࠊ⫈ぬ่⃭࡟ࡼࡾࠊ≉ᐃࡢᅗᙧࢆ㐃᝿ࡍࡿグ᠈ࡀ㞀ᐖࡉࢀ࡚࠸ࡿࡇ࡜ࡀุࡗࡓࠋ⫈ぬ่⃭࡜ᅗᙧ่⃭ࡢ㐃᝿ࡀᡂ❧ࡋࡓ࣐࢘ࢫ࡟ࠊ⫈ぬ่⃭ࢆ⾜࠺࡜ࠊ⫈ぬ㔝ࡢࡳ࡞ࡽࡎࠊࡑࡢ⫼ഃ㒊࡟㞄᥋ࡋࡓどぬ㔝࡟ࡶάືࡀグ㘓ࡍࡿࡇ࡜࠿ࡽࠊ⫈ぬ㔝ࡢ⫼ഃ㸦どぬ㔝ࡢ⭡ഃ㒊㸧ࡀ㐃᝿グ᠈ࡸᙧ≧ㄆ▱ࡢ㈐௵㒊఩࡛࠶ࡿ࡜ᛮࢃࢀࡿࠋ㐃᝿่⃭ࢆ⏝࠸ࡓሙྜࡢࡇࡢ㒊఩ࡢάືࡀF3FGKD࣐࢘ࢫ࡛㞀ᐖࡉࢀࡿࡇ࡜ࢆ☜ㄆࡋࡓࠋ௨ୖࡢ⤖ᯝ࠿ࡽࠊ⫈ぬ㔝ࡢ⫼ഃ࡟㞄᥋ࡍࡿどぬ㔝ࡀᙧ≧ㄆ▱࡟㔜せ࡞㒊఩࡛࠶ࡿ࡜ᛮࢃࢀࡿࠋ༶ࡕF3FGKD࣐࢘ࢫ࡛ࡣࠊ
ᚑ᮶ሗ࿌ࡋࡓࡼ࠺࡟⫼ഃ⤒㊰ࡢ㞀ᐖࡔࡅ࡛࡞ࡃࠊ⭡ഃ⤒㊰ࡢᶵ⬟ࡶ㞀ᐖࡉࢀ࡚࠸ࡓࠋ༶ࡕࠊどぬ⣔㐃ྜ㔝࡟ࡣF3FGKDࡢศᏊከᵝᛶ࡟౫Ꮡࡍࡿఱࡽ࠿ࡢ⚄⤒ᅇ㊰ࡀᏑᅾࡋࠊከᙬ࡞㧗ḟᶵ⬟ࢆᨭ࠼࡚࠸ࡿྍ⬟ᛶࡀ♧၀ࡉࢀࡿࠋϪ ㄽᩥ㸦ཎⴭࠊ⥲ㄝࠊ⑕౛ሗ࿌ࢆ༊ูࡋ࡞࠸㸧(1) Hishida R, Kudoh M, Shibuki K, Multimodal cortical sensory pathways revealed by sequential
transcranial electrical stimulation in mice. Neurosci. Res. 87, 49-55, 2014.
(2) Tohmi M, Meguro R, Tsukano H, Hishida R, Shibuki K, The extrageniculate visual pathway generates
distinct response properties in the higher visual areas of mice. Current Biology 24, 587-597, 2014.
ϫ ඹྠ◊✲   ◊✲㢟┠ࠕࣉࣟࢺ࢝ࢻ࣊ࣜࣥࡢ⬻ᶵ⬟ࠖ   ◊✲ෆᐜ⚄⤒≉␗ⓗ࠿ࡘከᵝᛶࢆ᭷ࡍࡿ⣽⬊᥋╔ᅉᏊࡢࣉࣟࢺ࢝ࢻ࣊ࣜࣥࡀ࡝ࡢࡼ࠺࡞
        ⬻ᶵ⬟࡟㛵୚ࡍࡿ࠿ࢆゎᯒࡍࡿࠋ   ཧຍᶵ㛵 ኱㜰኱Ꮫ ◊✲㢟┠ࠕ኱⬻⓶㉁㹌㹋㹂㸿ཷᐜయࡢᶵ⬟ࠖ   ◊✲ෆᐜ ኱⬻⓶㉁≉␗ⓗ࡟㹌㹋㹂㸿ཷᐜయᶵ⬟ࡀ༙ῶࡋ࡚࠸ࡿ㑇ఏᏊᨵኚ࣐࢘ࢫࢆ⏝࠸ࠊ   ኱⬻⓶㉁㹌㹋㹂㸿ཷᐜయࡀ࡝ࡢࡼ࠺࡞⤒㦂౫Ꮡⓗྍረᛶࡸ⬻ᶵ⬟࡟㛵ࢃࡿࡢ࠿  ࢆゎᯒࡍࡿࠋ   ཧຍᶵ㛵 㑇ఏᏛ◊✲ᡤ ◊✲㢟┠ ࠕ኱⬻⓶㉁ᢚไࢽ࣮ࣗࣟࣥࡢᶵ⬟ࠖ◊✲ෆᐜᢚไᛶࢽ࣮ࣗࣟࣥ࡟≉␗ⓗ࡟㹅㹄㹎ࢆⓎ⌧ࡍࡿ࣐࢘ࢫࢆ⏝࠸ࠊ኱⬻⓶㉁ࡢᢚไ
 ᛶࢽ࣮ࣗࣟࣥࡀ࡝ࡢࡼ࠺࡞⤒㦂౫Ꮡⓗྍረᛶࡸ⬻ᶵ⬟࡟㛵ࢃࡿࡢ࠿ࢆゎᯒࡍࡿࠋ   ཧຍᶵ㛵 ⩌㤿኱Ꮫ㸫㸫

∛ℂቇಽ㊁࠺ࠫ࠲࡞කቇಽ㊁㧔⛔ว⣖ᯏ⢻⎇ⓥ࠮ࡦ࠲࡯㧕⣖∔ᖚᮡᧄ⾗Ḯ⸃ᨆቇಽ㊁㧔↢๮⑼ቇ࡝࠰࡯ࠬ⎇ⓥ࠮ࡦ࠲࡯㧕 Σ ⎇ⓥ⚵❱㧔᭴ᚑຬ ᐔᚑᐕ᦬ᣣ⃻࿷㧕 Σ ∛ℂቇಽ㊁ᢎ ᝼  㜞ᯅ  ဋ     ᛛⴚ⡯ຬਤ↰ᥓᕺሶಎᢎ᝼  ⼾ፉ 㕏ሶ               ỘᎹ ᘕ๋    ഥ ᢎ  ઁ↰ ⌀ℂ㜞ፒ 㗅ሶഥ ᢎ  ᷡ᳓  ብ               ᢪ⮮ ᤐ⟤                             ⮮↰ ⍮ሶ                   ੐ോ⡯ຬ      ↸↰ਃ଻ሶ                             ਄↰ ᵗሶ                   ᄢቇ㒮ඳ჻⺖⒟   ┻ౝ ੫ሶ㧔␹⚻ౝ⑼㧕 ዊୖ ⦟੺
㧔⣖␹⚻ᄖ⑼㧕 ᮮጊ ⵨৻
㧔ಽሶ♖␹කቇ㧕 ᧁ᧛ ᱜᔒ                            㧔੩ㇺᐭ┙ක⑼ᄢቇ࡮␹⚻ౝ⑼ቇ㧕 ↰ਛ ⧷ᥓ
㥲⮮ ℂᕺ                             㧔␹⚻ౝ⑼㧕           ૒⮮ ᦮ᳯ 㧔␹⚻ౝ⑼㧕ᷡኅ ዏᒾ                             㧔␹ᚭᄢቇ࡮␹⚻ౝ⑼ቇ㧕                      ᄖ࿖ੱ⇐ቇ↢ ᒛ ⅎ Σ ࠺ࠫ࠲࡞කቇಽ㊁㧔⛔ว⣖ᯏ⢻⎇ⓥ࠮ࡦ࠲࡯㧕    ᢎ ᝼ ᩑ↰ ᣿⟤    ⣖∔ᖚᮡᧄ⾗Ḯ⸃ᨆቇಽ㊁㧔↢๮⑼ቇ࡝࠰࡯ࠬ⎇ⓥ࠮ࡦ࠲࡯㧕    ᢎ ᝼㧔౗㧕ᩑ↰ ᣿⟤Τ ⎇ⓥᵴേ    ∛ℂቇಽ㊁ߣ࠺ࠫ࠲࡞කቇಽ㊁߅ࠃ߮⣖∔ᖚᮡᧄ⾗Ḯ⸃ᨆቇಽ㊁ߪ‫౒ޔ‬หߢၮ␆ߣ⥃ᐥߩⲢวߩਅ‫↢ޔ‬ᬌ࡮೬ᬌߦ┙⣉ߒߚ‫ੱޟ‬૕␹⚻∛ℂቇ‫ࠍޠ‬ታ〣ߒߡ޿ࠆ‫∛ޕ‬ℂ⸃೬ߪ‫ᤨޔ‬㑆ᣣ
૕೙ߢⴕߥߞߡ߅ࠅ‫࠻ࡅޔ‬⣖⑼ቇߩ⎇ⓥ⊒ዷߦ⾗ߔࠆ⣖␹⚻∔ᖚᮡᧄ࡝࠰࡯ࠬߩ㊂⊛‫⾰ߟ߆ޔ‬⊛ߥలታߦദ߼ߡ޿ࠆ‫ޕ‬    ⎇ⓥኻ⽎ߦߪ‫ޔ‬ฦ⒳␹⚻ᄌᕈ∔ᖚ‫ޔ‬⣖ߩ⊒↢ߩࡔࠞ࠾࠭ࡓߣߘߩ⇣Ᏹ‫ޔ‬⣖⣲≌‫ޔ‬⣖ⴊ▤㓚ኂ‫ޔ‬⣕㜑ᕈ∔ᖚ‫ߦࠄߐޔ‬ਛᲥ࡮ઍ⻢࡮Ἳ∝ᕈ∔ᖚߥߤ߇޽ࠆ‫ޕ‬⣖␹⚻∔ᖚߩᄙ᭽ᕈߦᔕߓߚᬌ⚝ࠍ㸫㸫ၮ⋚ߦ⥃ᐥ∛ℂቇ⊛⎇ⓥࠍⴕ߁ߣߣ߽ߦ‫ޔ‬ේ࿃࡮ᯏᐨߩ⸃᣿ࠍᜰะߒߚਥዉ⊛‫ޔ‬ᡰេ⊛౒ห⎇
ⓥߦขࠅ⚵ࠎߢ޿ࠆ‫ޕ‬    ߥ߅‫ޔ‬ᐔᚑ㧞㧢ᐕᐲߦ߅ߌࠆ⎇ⓥᚑᨐߦߟ޿ߡߪ‫ޔ‬એਅߩ⊒⴫⺰ᢥߩႎ๔ߦᆔߨࠆߎߣߣߔ
ࠆ‫ޕ‬


Υ ⺰ᢥ㧔ේ⪺‫଀∝ޔ⺑✚ޔ‬ႎ๔ࠍ඙೎ߒߥ޿㧕

1.

૒⮮๺ᒾ㧘ၳᳯᱜ↵㧘┻ᨋᶈ⑲㧘㜞ᯅ ဋ㧘ᩑ↰᣿⟤ࡅ࠻⣖∛ℂಾ ߦ߅ߌࠆ in situ hybridization ᴺࠍ೑↪ߒߚ࠾ࡘ࡯ࡠࡦ߅ࠃ߮ࠣ࡝ࠕ⚦⢩ߩหቯ㧚ᣂẟකቇળ㔀⹹ 2014; 128 (12): 625-634㧚

2.

Wakabayashi K, Mori F, Kakita A, Takahashi H, Utsumi J, Sasaki H. Analysis of micro RNA fromarchived formalin-fixed paraffin-embedded samples of amyotrophic lateral sclerosis. Acta Neuropathol
Commun 2014; 2 (1): 173.

3.

⍹ේᥓᒾ㧘ᩑ↰᣿⟤㧘㜞ᯅ ဋ㧘ዊ㊁ኹ ℂ㧘⷏Ỉᱜ⼾㧚#.5 ߦ߅ߌࠆ URNKEGQUQOG ⇣Ᏹ⥃ᐥ␹⚻

2014; 54 (12): 1155-1157.
4.

ᩑ↰᣿⟤ࡅ࠻ߡࠎ߆ࠎ∛Ꮍߦ߅ߌࠆࠣ࡝ࠕ⚦⢩ߩ∛ℂ⚵❱ቇ⊛ᚲ⷗㧚⥃ᐥ␹⚻ 2014; 54 (12): 1136-

1138.
5. Natsumeda M, Igarashi H, Nomura T, Ogura R, Tsukamoto Y, Kobayashi T, Aoki H, Okamoto K,
Kakita A, Takahashi H, Nakada T, Fujii Y. Accumulation of 2-hydroxyglutarate in gliomas correlateswith survival: a study by 3.0-tesla magnetic resonance spectroscopy. Acta Neuropathol Commun 2014;
2 (1): 158.
6. ⼾ፉ㕏ሶᮮጊ⵨৻㜞ᯅ ဋ Pallido-nigro-luysian atrophy. Clin Neurosci 2014; 32: 1210-1211
7. Horie M, Watanabe K, Bepari AK, Nashimoto J, Araki K, Sano H, Chiken S, Nambu A, Ono K,
Ikenaka K, Kakita A, Yamamura K, Takebayashi H. Disruption of actin-binding domain-containing
Dystonin protein causes dystonia musculorum in mice.
8. Eur J Neurosci 2014; 40 (10): 3458-3471.
9. Korematsu S, Miyahara H, Kakita A, Izumi T. Elevated serum anti-phosphatidylcholine IgG antibody
in patients with influenza vaccination-associated optic neuritis. Vaccine 2014; 32 (48): 6345-6348.
10. ർᶆᒄ᮸‫ޔ‬ᩑ↰᣿⟤⚿▵ᕈ⎬ൻ∝㧚Epilepsy 2014; 8 (2): 74-76.
11. ᩑ↰᣿⟤㧚ዊఽߡࠎ߆ࠎේᕈ∛Ꮍߩᄖ⑼∛ℂ㧚No To Hattatsu 2014; 46 (6): 413-417.
12. Konno T, Tada M, Shiga A, Tsujino A, Eguchi H, Masuda-Suzukake M, Hasegawa M, Nishizawa M,
Onodera O, Kakita A, Takahashi H. C9ORF72 repeat-associated non-ATG-translated polypeptides are
distributed independently of TDP-43 in a Japanese patient with c9ALS. Neuropathol Appl Neurobiol
2014; 40 (6): 783-788.
13. Ota K, Obayashi M, Ozaki K, Ichinose S, Kakita A, Tada M, Takahashi H, Ando N, Eishi Y,
Mizusawa H, Ishikawa K. Relocation of p25ǂ /tubulin polymerization promoting protein from thenucleus to the perinuclear cytoplasm in the oligodendroglia of sporadic and COQ2 mutant multiple
system atrophy. Acta Neuropathol Commun 2014; 2 (1): 136.
14. Murakami T, Morimoto J, Hoshi A, Nakatani-Enomoto S, Ichikawa M, Tasaki K, Ogura R,
Toyoshima Y, Kakita A, Saito K, Takahashi H, Ugawa Y. Cerebral amyloid angiopathy-related
leukoencephalopathy: successful steroid treatment for neurological deficits and subcortical white matterlesions partly involving the cortical gray matter. Neurology and Clinical Neuroscience 2014; 2 (4): 119121.
㸫㸫

15. Miyashita A, Hatsuta H, Kikuchi M, Nakaya A, Saito Y, Tsukie T, Hara N, Ogishima S, Kitamura N,
Akazawa K, Kakita A, Takahashi H, Murayama S, Ihara Y, Ikeuchi T, Kuwano R, Japanese
Alzheimer’s Disease Neuroimaging Initiative. Genes associated with the progression of neurofibrillarytangles in Alzheimer’s disease. Translational Psychiatry 2014; 4: e396.
16. Kuga Y, Ohnishi H, Kodama Y, Takakura S, Hayashi M, Yagi R, Fukutome K, Matsushima K,
Okamoto K, Taomoto K, Takahashi H. Cerebral and spinal cord tanycytic ependymomas in a young
adult with a mutation in the NF2 gene. Neuropathology 2014; 34:406-413
17. Kimura T, Jiang H, Konno T, Seto M, Iwanaga K, Tsujihata M, Satoh A, Onodera O, Kakita A,
Takahashi H. Bunina bodies in motor and non-motor neurons revisited: a pathological study in an ALS
patient after long-term survival on a respirator. Neuropathology 2014; 34 (4): 392-397.
18. Miki Y, Mori F, Tanji F, Kurotaki H, Kakita A, Takahashi H, Wakabayashi K. An autopsy case ofincipient Pick’s disease: immunohistochemical profiles of early stage Pick body formation.
Neuropathology 2014; 34 (4): 386-391.
19. Kunii Y, Miura I, Matsumoto J, Hino M, Wada A, Niwa SI, Nawa H, Sakai M, Someya T, Takahashi
H, Kakita A, Yabe H. Elevated postmortem striatal t-DARPP expression in schizophrenia andassociations with DRD2/ANKK1 polymorphism. Progress in Neuro-Psychopharmacology &Biological Psychiatry. Prog Neuropsychopharmacol Biol Psychiatry 2014; 53: 123-128.
20. ᩑ↰᣿⟤㧚߮߹ࠎᕈࡔ࡜࠾ࡦ⚦⢩Ⴧᱺ∝㧘㜑⤑㤥⦡⣲∝㧚(In) ␹⚻∝୥⟲ IIĬߘߩઁߩ␹⚻∔ᖚࠍ฽߼ߡ̆㧚೎ౠᣣᧄ⥃ῼ ᣂ㗔ၞ೎∝୥⟲ࠪ࡝࡯࠭No. 28. ᣣᧄ⥃ῼ㧘᧲੩㧚 ᐕ  ᦬  ᣣ㧚pp. 307310 (total 883p)㧚
21. Nakahara A, Yoshida T, Yazawa M, Ehara T, Nakayama J, Kakita A, Ogura R, Asakawa M, SuzukiKouyama E, Oyanagi K. “Gliomatosis encephali” as a novel category of brain tumors by the first
autopsy case report of gliomatosis cerebelli. Neuropathology 2014; 34 (3): 295-303.
22. Tanji K, Miki Y, Ozaki T, Murayama A, Yoshida H, Mimura J, Matsumiya T, Mori F, Imaizumi T,
Ihoh K, Kakita A, Takahashi H, Wakabayashi K. Phosphorylation of serine 349 of p62 in Alzheimer’s
disease brain. Acta Neuropathol Commun 2014; 2 (1): 50.
23. Del Bigio MR, Hainfellner JA, McLean CA, Powell SZ, Sikorska B, Takahashi H, Weis J, Xuereb JH.
Neuropathology training worldwide-evolution and comparisons. Brain Pathol 2014; 24: 285-298
24. Ogura R, Yoshimura J, Sano M, Kawasaki S, Nishiyama K, Okamoto K, Takahashi H, Fujii Y, Kakita
A. Entrapment of the inferior horn of the lateral ventricle with enlargement of the bilateral choroid
plexus. Neuropathology 2014; 34 (2): 210-213.
25. Sato T, Ouchi H, Shimbo J, Sato A, Yamazaki M, Hashidate H, Igarashi S, Kakita A. Coexistence of
amyotrophic lateral sclerosis with neuro-Behçet’s disease presenting as a longitudinally extensive
spinal cord lesion: clinicopathologic features of an autopsied patient. Neuropathology 2014; 34 (2):185-189.
26. Miki Y, Mori F, Kon T, Tanji K, Toyoshima Y, Yoshida M, Sasaki H, Kakita A, Takahashi H,
Wakabayashi K. Accumulation of sigma-1 receptor is common to neuronal nuclear inclusions invarious neurodegenerative diseases. Neuropathology 2014; 34 (2): 148-158.
27. Mori F, Toyoshima Y, Tanji K, Kakita A, Takahashi H, Wakabayashi K. FUS co-localizes withpolyglutamine, but not with TDP-43 in neuronal intranuclear inclusions in spinocerebellar ataxia type 2.
Neuropathol Appl Neurobiol 2014; 40 (3): 351-355.
28. Matsushima A, Yoshida T, Yoshida K, Ohara S, Toyoshima Y, Kakita A, Ikeda S. Superficial siderosis
associated with aceruloplasminemia. Case report. J Neurol Sci 2014; 339 (1-2): 231-234.
㸫㸫

29. ᩑ↰᣿⟤㧚ߡࠎ߆ࠎේᕈߩ∛ℂ㧚╙  ┨ߡࠎ߆ࠎߩ∛࿃࡮∛ᘒ↢ℂ㧚(In) ᣣᧄߡࠎ߆ࠎቇળ㧔✬㓸㧕ߡࠎ߆ࠎኾ㐷කࠟࠗ࠼ࡉ࠶ࠢ㧚̆ߡࠎ߆ࠎߦ߆߆ࠊࠆකᏧߩߚ߼ߩၮ␆⍮⼂̆㧚⸻ᢿߣᴦ≮␠㧘᧲੩㧚

2014 ᐕ 3 ᦬ 25 ᣣ㧚pp. 27-30 (total 303p).
30. Tomonoh Y, Deshimaru M, Araki K, Miyazaki Y, Arasaki T, Tanaka Y, Kitamura H, Mori F,
Wakabayashi K, Yamashita S, Saito R, Ihoh M, Uchida T, Yamada J, Migita K,, Ueno S, Kitaura H,
Kakita A, Lossin C, Takano Y, Hirose S. The kick-in system: a novel rapid knock-in strategy. PLoS
One 2014; 9 (2): e88549.
31. 㜞ᯅ ဋේ⊒ᕈ஥⚝⎬ൻ∝೎ౠᣣᧄ⥃ᐥᣂ㗔ၞ೎∝୥⟲ࠪ࡝࡯࠭ No. 27 ␹⚻∝୥⟲㧔╙  㧕(II)֣ߘߩઁߩ␹⚻∔ᖚࠍ฽߼ߡ֣, pp 525-529
32. Ouchi H, Toyoshima Y, Tada M, Oyake M, Aida I, Tomita I, Satoh A, Tsujihata M, Takahashi H,
Nushizawa M, Shimohata T. Pathology and sensitivity of current clinical criteria in corticobasal
syndrome. Mov Disord 22014; 29: 238-244
33. Yokoseki A, Saji E, Arakawa M, Kosaka T, Hokari M, Toyoshima Y, Okamoto K, Takeda S, Sanpei
K, Kikuchi H, Hirohata S, Akazawa K, Kakita A, Takahashi H, Nishizawa M, Kawachi I.
Hypertrophic pachymeningitis: significance of myeloperoxidase anti-neutrophil cytoplasmic antibody.
Brain 2014; 137 (Pt2): 520-536.
34. Mori F, Kakita A, Takahashi H, Wakabayashi K. Co-localization of Bunina bodies and TDP-43
inclusions in lower motor neurons in amyotrophic lateral sclerosis. Neuropathology 2014; 34 (1): 71-76.
35. Kon T, Mori F, Yanji F, Miki Y, Toyoshima Y, Yoshida M, Sasaki H, Kakita A, Takahashi H,
Wakabayashi K. ALS-associated protein FIG4 is localized in Pick and Lewy bodies, and neuronal
nuclear inclusions in polyglutamine and intranuclear inclusion body diseases. Neuropathology 2014; 34(1): 19-26.
36. Miyahara H, Natsumeda M, Yoshimura J, Okazaki K, Toyoshima Y, Fujii Y, Takahashi H, Kakita A.
Neuronal differentiation associated with Gli3 expression predicts favorable outcome in patients withmedulloblastoma. Neuropathology 2014; 34 (1): 1-10.
37. Miyashita A, Wen Y, Kitamura N, Matsubara E, Kawarabayashi T, Shoji M, Tomita N, Furukawa K,
Arai H, Asada T, Harigaya Y, Ikeda M, Amari M, Hanyu H, Higuchi S, Nishizawa M, Suga M,
Kawase Y, Akatsu H, Imagawa M, Hamaguchi T, Yamada M, Morihara T, Takeda M, Takao T,
Nakata K, Sasaki K, Watanabe K, Nakashima K, Urakami K, Ooya T, Takahashi M, Yuzuhira T,
Serikawa K, Yoshimoto S, Nakagawa R, Saito Y, Htsuta H, Murayama S, Kakita A, Takahashi H,
Yamaguchi H, Akazawa K, Kanazawa I, Ihara Y, Ikeuchi T, Kuwano R. Lack of genetic association
between TREM2 and late-onset Alzheimer’s disease in a Japanese population. J Alzheimers Dis 2014;41 (4): 1031-1038.
38. Liu J, Yoneoka Y, Tanaka K, Satou H, Abe E, Watanabe N, Fujii Y, Takahashi H, Aoyama H.
Pituitary intratumoral hemorrhage during radiation therapy following partial removal of giant pituitaryadenoma: a case report. Case Rep Clin Med 2014; 3: 38-41
39. Konno T, Tada M, Tada M, Koyama A, Nozaki H, Harigaya Y, Nishimiya J, Matsunaga A, Yoshikura
N, Ishihara K, Arakawa M, Isami A, Okazaki K, Yokoo H, Ihoh K, Yoneda M, Kawamura M, Inuzuka
T, Takahashi H, Nishizawa M, Onodera O, Kakita A, Ikeuchi T. Haploinsufficiency of CSF-1R andclinicopathological characterization in patients with HDLS. Neurology 2014; 82 (2): 139-148.
40. Bundo M, Toyoshima M, Ueda J, Nemoto-Miyauchi T, Sunaga F, Toritsuka M, Ikawa D, Kakita A,
Okada Y, Akamatsu W, Kato M, Okano H, Kasai K, Kishimoto T, Nawa H, Yoshikawa T, Kato T,
Iwamoto K. Increased L1 retrotransposition in the neuronal genome in schizophrenia. Neuron 2014; 81(1): 1-8.
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41. Tatsumi S, Mimuro M, Iwasaki Y, Takahashi R, Kakita A, Takahashi H, Yoshida M. Argyrophilic
grains are reliable disease-specific features of corticobasal degeneration. J Neuropathol Exp Neurol
2014; 73 (1): 30-38.
42. ૒⮮᥏‫ޔ‬㈬੗⋥ሶ‫ޔ‬ᣂ଻ᷕテ‫ޔ‬ᯅ┙⧷᮸‫ޔ‬੖ච፲ୃ৻‫ޔ‬ᩑ↰᣿⟤‫ޔ‬ጊፒర⟵㧚⪺᣿ߥ╭∨ᡨ࡮✢⛽᧤ᕈ෼❗ࠍ઻޿᛫ VGKC ⶄว૕᛫૕߇㜞୯ߢ޽ߞߚ╭⪜❗ᕈ஥⚝⎬ൻ∝ߩ㧝೬ᬌ଀㧚⥃ᐥ␹⚻2014; 54 (1):32-37.

Φ ౒ห⎇ⓥ   ᧄ∛ℂቇಽ㊁࡮࠺ࠫ࠲࡞කቇಽ㊁ߪ‫ޔ‬ᢥㇱ⑼ቇ⋭⹺ቯߩᣂẟᄢቇ⣖⎇ⓥᚲ‫ޟ‬⣖␹⚻∛ℂᮡᧄ⾗Ḯᵴ↪ߩవ┵⊛౒ห⎇ⓥ᜚ὐ‫ߩޠ‬ਛᩭಽ㊁ߣߒߡ‫࠻ࡅޔ‬⣖⑼ቇߦ㑐ߔࠆࡊࡠࠫࠚࠢ࠻ဳ߅ࠃ
߮ㅪ៤⾗Ḯ೑↪ဳߩ࿖ౝ౒ห⎇ⓥࠍផㅴߒߡ޿ࠆ‫ޕ‬   ⎇ⓥ㗴⋡  ‫⚻␹ޟ‬ᄌᕈ∔ᖚߦ㑐ߔࠆ␹⚻∛ℂቇ⊛⎇ⓥ‫ޠ‬   ⎇ⓥౝኈ  ␹⚻ᄌᕈ∔ᖚ‫ߦߊߣޔ‬ㅴⴕᕈᩭ਄ᕈ㤗∽㧔࠲࠙ࠝࡄ࠴࡯㧕‫∛ࡦ࠰ࡦࠠ࡯ࡄޔ‬㧔ࠪ࠿ࠢ࡟ࠗࡁࡄ࠴࡯㧕߿╭⪜❗ᕈ஥⚝⎬ൻ∝(TDP-43ࡊࡠ࠹ࠗࡁࡄ࠴࡯ߩ⥃ᐥ∛ℂ߿∛࿃⸃᣿ߦㅪߥࠆ౒ห⎇ⓥࠍⴕߥߞߡ޿ࠆ‫ޕ‬   ෳടᯏ㑐  ᒄ೨ᄢቇ‫᧲ޔ‬੩ㇺකቇ✚ว⎇ⓥᚲ‫ޔ‬ጘ㒂ᄢቇ࡮ጘ㒂⮎⑼ᄢቇ
           ାᎺᄢቇ‫᧲ޔ‬੩ᅚሶක⑼ᄢቇ‫ޔ‬ᗲ⍮ක⑼ᄢቇ‫ޔ‬੩ㇺᄢቇ ⎇ⓥ㗴⋡  ‫ޟ‬㔍ᴦߡࠎ߆ࠎේᕈ∛Ꮍߦ㑐ߔࠆᄖ⑼∛ℂᮡᧄߩ⸃ᨆ‫ޠ‬   ⎇ⓥౝኈ  㔍ᴦߡࠎ߆ࠎේᕈ∛Ꮍߩ∛ᘒᒻᚑᯏᐨߩ⸃᣿ࠍ⋡⊛ߦ‫ޔ‬ฦ⒳∛ᘒ㧔㒢ዪᕈ⊹⾰ᒻᚑ⇣Ᏹ‫▵⚿ޔ‬ᕈ⎬ൻ∝ߥߤ㧕ߩಾ㒰⣖⚵❱ࠍ↪޿ߚ∛ℂ⚵❱ቇ⊛‫↢ޔ‬ൻቇ⊛‫↢ޔ‬ℂቇ⊛⸃ᨆࠍㅴ߼ߡ޿ࠆ‫ޕ‬  ෳടᯏ㑐  ࿖┙∛㒮ᯏ᭴⷏ᣂẟਛᄩ∛㒮‫ޔ‬࿖┙♖␹࡮␹⚻ක≮⎇ⓥ࠮ࡦ࠲࡯∛㒮‫ޔ‬      ᐢፉᄢቇ‫ޔ‬࿖┙ᚑ⢒ක≮࠮ࡦ࠲࡯∛㒮 ⎇ⓥ㗴⋡  ‫∔⚻␹␹♖ޟ‬ᖚߩಽሶ∛ℂቇ⊛⸃ᨆ‫ޠ‬   ⎇ⓥౝኈ  ♖␹␹⚻∔ᖚߩ೬ᬌ⣖ࠍኻ⽎ߣߒߚ⥃ᐥ∛ℂ‫ޔ‬෸߮ಽሶ∛ℂቇ⊛∛ᘒ⸃ᨆߩߚ߼ߩಓ⚿⣖ᮡᧄ⾗Ḯࠍឭଏߔࠆߎߣߢ‫∔⚻␹␹♖ޔ‬ᖚ‫⛔ߦߊߣޔ‬วᄬ⺞∝ߩ∛ᘒᒻᚑᯏᐨߩ⸃ᨆࠍㅴ߼ߡ޿ࠆ‫ޕ‬   ෳടᯏ㑐  ╳ᵄᄢቇ‫⑔ޔ‬ፉ⋵┙ක⑼ᄢቇ‫ޔ‬ℂൻቇ⎇ⓥᚲ㸫㸫ಽሶ∛ᘒቇ㧔ቴຬ㧕ಽ㊁ Σ ⎇ⓥ⚵❱㧔᭴ᚑຬ ᐔᚑᐕ᦬ᣣ⃻࿷㧕   ᢎ ᝼㧔૬㧕  ⧯ᨋ ቁ৻   ಎᢎ᝼㧔૬㧕  ᫪ ᢥ⑺
Τ ⎇ⓥᵴേ

ᒰಽ㊁ߢߪ‫⚻␹ޔ‬㔍∛ߩ∛ᘒ⸃᣿ࠍ⋡ᮡߦ‫∛ޔ‬ℂᒻᘒቇ‫ޔ‬ಽሶ↢‛ቇ‫∛ޔ‬ᘒ↢ൻቇߥߤߩᚻᴺࠍ↪
޿⎇ⓥࠍㅴ߼ߡ޿ࠆ‫⚻␹ޕ‬ᄌᕈ∔ᖚߩᄙߊߪ࠲ࡦࡄࠢ⾰⫾Ⓧ∛ߢ޽ࠆߎߣ߆ࠄ‫ޔ‬‫⚿ߩ⾰ࠢࡄࡦ࠲ޟ‬ว࡮ୃ㘼࡮ಽ⸃‫ⷰߩޠ‬ὐ߆ࠄࠕࡊࡠ࡯࠴ࠍⴕߞߡ޿ࠆ‫ޟޔߦࠄߐޕ‬ኽ౉૕ᒻᚑ‫⚦⚻␹ޟ߿ޠ‬⢩ᱫ‫ߥߢߌߛޠ‬ߊ‫ߦ⃻⊒ߩ⁁∝⚻␹ޔ‬㊀ⷐߥㇱ૏ߣߒߡ‫ߩޠࠬࡊ࠽ࠪޟ‬ᄌൻߦ߽ὶὐࠍᒰߡߡ޿ࠆ‫ޕ‬⃻࿷ߩ⎇ⓥ࠹࡯ࡑߪ‫ޔ‬㧝㧕␹⚻ᄌᕈ∔ᖚߦ߅ߌࠆኽ౉૕ᒻᚑࡔࠞ࠾࠭ࡓ‫ޔ‬㧞㧕ࠣ࡝ࠕ⚦⢩ߩᯏ⢻ߣฦ⒳∛ᘒߦ߅ߌࠆᄌൻ‫ޔ‬㧟㧕ㆮવሶᡷᄌࡕ࠺࡞േ‛ࠍ↪޿ߚ∛ᘒ⸃ᨆߢ޽ࠆ‫ࡄࡁࠗ࡟ࠢ࠿ࠪޔߦ․ޕ‬࠴࡯㧔ࡄ࡯ࠠࡦ࠰ࡦ∛‫࡯ࡆ࡟ޔ‬ዊ૕ဳ⹺⍮∝‫ޔ‬ᄙ♽⛔⪜❗∝㧕߿ࡐ࡝ࠣ࡞࠲ࡒࡦ∛ߩ೬ᬌ⣖⚵❱ࠍ↪
޿ߚ⎇ⓥߪ∛ℂቇಽ㊁߿⣖∔ᖚᮡᧄ⾗Ḯ⸃ᨆቇಽ㊁ߣ౒หߢㅴ߼ߡ޿ࠆ‫ ޕ‬Υ ⺰ᢥ㧔ේ⪺‫଀∝ޔ⺑✚ޔ‬ႎ๔ࠍ඙೎ߒߥ޿㧕
1.

Kon T, Mori F, Tanji K, Miki Y, Toyoshima Y, Yoshida M, Sasaki H, Kakita A, Takahashi H, Wakabayashi K. ALSassociated protein FIG4 is localized in Pick and Lewy bodies, and also neuronal nuclear inclusions, in polyglutamine andintranuclear inclusion body diseases. Neuropathology 34: 19-26, 2014

2.

Mori F, Kakita A, Takahashi H, Wakabayashi K. Co-localization of Bunina bodies and TDP-43 inclusions in lower motor
neurons in amyotrophic lateral sclerosis. Neuropathology 34: 71-76, 2014

3.

Mori F, Toyoshima Y, Tanji K, Kakita A, Takahashi H, Wakabayashi K. FUS co-localizes with polyglutamine, but not with
TDP-43 in neuronal intranuclear inclusions in spinocerebellar ataxia type 2. Neuropathol Appl Neurobiol 40: 351-355, 2014

4.

Miki Y, Tomiyama M, Kurotaki H, Wakabayashi K, Baba M. Primary central nervous system lymphoma mimicking
Bickerstaff’s encephalitis. Neurol Sci 35: 139-141, 2014

5.

Mori F, Watanabe Y, Miki Y, Tanji K, Odagiri S, Eto K, Wakabayashi K. Ubiquitin-negative, eosinophilic neuronalcytoplasmic inclusions associated with stress granules and autophagy: an immunohistochemical investigation of two cases.
Neuropathology 34: 140-147, 2014

6.

Miki Y, Mori F, Kon T, Tanji K, Toyoshima Y, Yoshida M, Sasaki H, Kakita A, Takahashi H, Wakabayashi K. Accumulationof the sigma-1 receptor is common to neuronal nuclear inclusions in various neurodegenerative diseases. Neuropathology
34: 148-158, 20147.

Miki Y, Mori F, Tanji K, Kurotaki H, Kakita A, Takahashi H, Wakabayashi K. An autopsy case of incipient Pick’s disease:
Immunohistochemical profile of early-stage Pick body formation. Neuropathology 34: 386-391, 2014

8.

Kobayashi T, Sugai M, Mori F, Wakabayashi K, Hakamada K. A morphometric investigation of pulmonary sympathetic
innervation in nitrofen-induced congenital diaphragmatic hernia rats. Hirosaki Med J 65: 74-81, 2014

9.

Tomonoh Y, Deshimaru M, Araki K, Miyazaki Y, Arasaki T, Tanaka Y, Kitamura H, Mori F, Wakabayashi K, Yamashita
S, Saito R, Itoh M, Uchida T, Yamada J, Migita K, Ueno S, Kitaura H, Kakita A, Lossin C, Takano Y, Hirose S. The kick-in
system: a novel rapid knock-in strategy. PLoS One 9: e88549, 2014㸫㸫

10. Kon T, Funamizu Y, Miki Y, Tomiyama M, Baba M, Kurotaki H, Wakabayashi K. An autopsy case of meningeal
carcinomatosis with parenchymal invasion through the cranial and spinal nerve roots. Neuropathology 34: 499-503, 2014
11. Tanji K, Miki Y, Ozaki T, Maruyama A, Yoshida H, Mimura J, Matsumiya T, Mori F, Imaizumi T, Itoh K, Kakita A,
Takahashi H, Wakabayashi K. Phosphorylation of serine 349 of p62 in Alzheimer’s disease brain. Acta Neuropathol Comm
2: 50, 201412. Sugeno N, Hasegawa T, Tanaka N, Fukuda M, Wakabayashi K, Oshima R, Konno M, Miura E, Kikuchi A, Baba T, Anan
T, Nakao M, Geisler S, Aoki M, Takeda A. K63-linked ubiquitination by E3 Ubiquitin Ligase Nedd4-1 facilitatesendosomal sequestration of internalized alpha-synuclein. J Biol Chem 289: 18137-18151, 2014
13. Miki Y, Tanji K, Mori F, Wakabayashi K. Sigma-1 receptor is involved in degradation of intranuclear inclusions in a cellularmodel of Huntington’s disease. Neurobiol Dis 74C: 25-31, 2014
14. Wakabayashi K, Mori F, Kakita A, Takahashi H, Utsumi J, Sasaki H. Analysis of microRNA from archived formalin-fixedparaffin-embedded specimens of amyotrophic lateral sclerosis. Acta Neuropathol Comm 2:173, 2014
15. Tanji K, Mori F, Wakabayashi K. The Role of Atg8 Homologue in Lewy Body Disease. In: Hayat MA, ed. Autophagy:
Cancer, Other Pathologies, Inflammation, Immunity, Infection, and Aging. Volume 2, Amsterdam, Elsevier; p. 383-389,
2014
16. ⧯ᨋቁ৻࡟ࡆ࡯ዊ૕∛ߦ߅ߌࠆ∛ᄌߩ᜛ᄢㅴዷᯏ᭴Dementia Japan 28: 11-17, 201417. ⧯ᨋቁ৻‫ޔ‬ਤᴦ㇌๺/5#ߣࠝ࡯࠻ࡈࠔࠫ࡯⥃ᐥ␹⚻54: 966-968, 2014Φ ౒ห⎇ⓥ   ⎇ⓥ㗴⋡  ‫⚻␹ޟ‬ᄌᕈ∔ᖚߦ߅ߌࠆ࡙ࡆࠠ࠴ࡦ㑐ㅪ࠲ࡦࡄࠢ⾰ߩ㑐ਈ‫ޠ‬   ⎇ⓥౝኈ ␹⚻ᄌᕈ∔ᖚ‫⇣ߦ․ޔ‬Ᏹ࠲ࡦࡄࠢ⾰ߩ⫾Ⓧ߿ಽ⸃ߦ࡙ࡆࠠ࠴ࡦ㑐ㅪ࠲ࡦࡄࠢ⾰㧔0'&&07$ߥߤ㧕߇ᨐߚߔᓎഀߦߟ޿ߡ‫ޔ‬೬ᬌ⣖⚵❱ࠍ↪޿⎇ⓥࠍㅴ߼ߡ޿ࠆ‫ޕ‬   ෳടᯏ㑐 ᒄ೨ᄢቇ⣖␹⚻∛ᘒ⎇ⓥᣉ⸳⣖␹⚻∛ℂቇ⻠ᐳ‫ࠕࠫ࡯࡚ࠫޔ‬ක⑼ᄢቇಽሶࠪࡖ           ࡍࡠࡦ࠮ࡦ࠲࡯

㸫㸫⣖␹⚻ᄖ⑼ቇಽ㊁ Σ ⎇ⓥ⚵❱㧔᭴ᚑຬ ᐔᚑᐕ᦬ᣣ⃻࿷㧕   ᢎ᝼   ⮮੗ᐘᒾ   ಎᢎ᝼  ጟᧄᶈ৻㇢‫⑔ޔ‬ᄙ⌀ผ   ഥᢎ   㕍ᧁ ᵗ     ඳ჻⺖⒟ᄢቇ㒮↢  ዊୖ⦟੺‫ޔ‬ጟ↰ᱜᐽ‫ޔ‬㋈ᧁ୶᣿‫⮮ޔ‬ේ⑲ర‫ޔ‬Ⴆᧄ૫ᐢΤ ⎇ⓥᵴേ
‫ޣ‬ၮ␆⎇ⓥ‫ޤ‬ ࠝ࡯࠻ࡈࠔࠫ࡯ߦᵈ⋡ߒߚᖡᕈ␹⚻⤔⣲ߦኻߔࠆᣂߚߥᴦ≮ᴺߩዷ㐿 /)/6 ᵴᕈ‫ޔ‬+&*OWVCVKQP ߩ⸃ᨆߦၮߠߊᖡᕈ␹⚻⤔⣲ߩ∛ᘒ⎇ⓥ ࡈ࡜ࡆࡦⰯశࠗࡔ࡯ࠫࡦࠣࠍ↪޿ߚߡࠎ߆ࠎ⊒૞⊒↢ᯏᐨߩ⸃᣿ ࡜࠶࠻⣖ᐙࠣ࡝ࠝ࡯ࡑࡕ࠺࡞ߦኻߔࠆ %'& EQPXGEVKQPGPJCPEGFFGNKXGT[ ᴺߦ㑐ߔࠆ⎇ⓥ వㅴ⊛  ᰴరᎿቇࠍ೑↪ߒߚ⣖␹⚻ᄖ⑼ᚻⴚࠪࡒࡘ࡟࡯࡚ࠪࡦ㧛࠻࡟࡯࠾ࡦࠣࠪࠬ࠹ࡓߩ㐿⊒࡮⥃ᐥᔕ↪ /WNVKRCTCOGVTKE/4+ ࠍ↪޿ߚ⯯ⴊᕈ⣖ⴊ▤㓚ኂߩ∛ᘒ⸃ᨆ 0GWTQOQFWNCVKQP ࠍ↪޿ߚᗵⷡㆇേ㊁⊹⾰ߦ߅ߌࠆᯏ⢻ઍఘᯏォߩ⸃᣿㧙H/4+ ࠍ↪޿ߚ⎇ⓥ ㄭ⿒ᄖಽశᴺࠍ↪޿ߚⴚ೨ᯏ⢻⹏ଔ‫ࠎ߆ࠎߡޔ‬ὶὐߩ∛ᘒ⸃ᨆ‫⥃ޣ‬ᐥ⎇ⓥ‫ޤ‬ ᖡᕈ␹⚻⤔⣲ 㑆⣖࡮ਅု૕⣲≌ ዊఽ⣖⣲≌‫ޔ‬వᄤᄸᒻ 㗡⬄ᐩ⣲≌ ᯏ⢻ᕈ∔ᖚ ⯯ⴊᕈ⣖ⴊ▤㓚ኂ ⣖േ⣂≜‫ޔ‬⣖േ㕒⣂ᄸᒻ ⣄ᬁ࡮⣄㜑∔ᖚΥ ⺰ᢥ㧔ේ⪺‫଀∝ޔ⺑✚ޔ‬ႎ๔ࠍ඙೎ߒߥ޿㧕1.

Natsumeda M, Igarashi H, Nomura T, Ogura R, Tsukamoto Y, Kobayashi T, Aoki H, Okamoto K, Kakita A, Takahashi H,
Nakada T, Fujii Y. Accumulation of 2-hydroxyglutarate in gliomas correlates with survival: a study by 3.0-tesla magnetic
resonance spectroscopy. Acta Neuropathol Commun. 2(1):158, 2014

2.

Fukuda M, Takao T, Hiraishi T, Yoshimura J, Yajima N, Saito A, Fujii Y. Clinical factors predicting outcomes after surgicalresection for sporadic cerebellar hemangioblastomas. World Neurosurg. 82(5):815-21, 20143.

Yoshimura J, Tsukamoto Y, Sano M, Hasegawa H, Nishino K, Saito A, Fukuda M, Okamoto K, Fujii Y. Successful
removal of a huge hypervascular tentorial cavernous angioma after preoperative endovascular embolization. J Neurosurg
Pediatr. 14(1):43-7, 2014㸫㸫4.

Harada A, Nishiyama K, Yoshimura J, Sano M, Fujii Y. Intraspinal lesions associated with sacrococcygeal dimples. J
Neurosurg Pediatr. 14(1):81-6, 2014

5.

Yuichiro Yoneoka, Naoto Watanabe, Mitsuo Kohno, Daisuke Satoh, Hitoshi Takahashi, Yukihiko Fujii. Technical note:
Endoscopic resection of a dermoid cyst anchored to the anterior optic chiasm. Interdisciplinary Neurosurgery 1(2) 21-5,
2014

6.

Yuichiro Yoneoka, Naoto Watanabe, Masayasu Okada, Kazuhiro Ando, Yukihiko Fujii Is Regression of Intracranial
Germinoma Spontaneous or Diagnostic Radiation-Induced? A Case Report. J Neurol Res. 4(1):41-8, 20147.

Nishikawa T, Okamoto K, Matsuzawa H, Terumitsu M, Nakada T, Fujii Y. Detectability of neural tracts and nuclei in thebrainstem utilizing 3DAC-PROPELLER. J Neuroimaging. 24(3):238-44, 20148.

Kurabe S, Itoh K, Matsuzawa H, Nakada T, Fujii Y. Expansion of sensorimotor cortical activation for unilateral handmotion during contralateral hand deafferentation. Neuroreport. 25(6):435-9, 2014

9.

Ogura R, Yoshimura J, Sano M, Kawasaki S, Nishiyama K, Okamoto K, Takahashi H, Fujii Y, Kakita A.. Entrapment of
the inferior horns of the lateral ventricle with enlargement of the bilateral choroid plexus. Neuropathology. 34(2):210-3, 2014.

Φ ౒ห⎇ⓥਛᨔ␹⚻ේ⊒ᖡᕈ࡝ࡦࡄ⣲ߩࡑࠗࠢࡠ40#⊒⃻⸃ᨆᣂẟᄢቇ⣖⎇ⓥᚲ ੩ㇺᐭ┙ක⑼ᄢቇ ජ⪲ᄢቇ ጊญᄢቇ

㸫㸫⚄⤒ෆ⛉Ꮫศ㔝Ϩ ◊✲⤌⧊㸦ᵓᡂဨ ᖹᡂᖺ᭶᪥⌧ᅾ㸧ᩍᤵ   す⃝ ṇ㇏    ෸ᩍᤵ   ୗ⏿ ாⰋ    ㅮᖌ   Ἑෆ Ἠ     ຓᩍ   㧗ᶫ ဴဢ    ຓᩍ    ㉥ᒾ 㟹ஂຓᩍ   ௚⏣ ṇ⩏    ຓᩍ    㔠⃝ 㞞ே≉௵ຓᩍ ሀ⏣ ៅ୍≉௵ຓᩍ ୰㔝 ோ⨾ᩆᛴ㒊 ≉௵ຓᩍ  ୖᮧ ᫀᐶᢏ⾡⫋ဨ 㔠Ꮚ ୕ὠᏊࠊᕝཱྀ ࡉࡸ࠿ࠊᑠᒣ ⨾ဏ༤ኈㄢ⛬኱Ꮫ㝔⏕
㡲㈅ ❶ᘯࠊᡭሯ ᩄஅࠊ▼ᕝ ṇ඾ࠊᵽཱྀ ┿ஓࠊ᭳⏣ Ἠࠊ㐲⸨ ᑑᏊࠊ➉ෆ ுᏊࠊ✑ⱉ ୓ᮤᏊࠊ㫽㇂㒊 ┿ྐࠊ┠ᓮ ┤ᐇࠊ୕ᾆ ೺ࠊୗ⏿ ᩗᏊࠊబ⸨ ᭸Ụࠊ㰻⸨ ⌮ᜨࠊᏱὠぢ ᏹኴࠊୖᮧ ᫀᐶಟኈㄢ⛬኱Ꮫ㝔⏕୕ᾆ ༡ࠊ⸨⏣ ⳯᦬ϩ ◊✲άື
࠙⬻᱾ሰ࡟ᑐࡍࡿ⾑⟶ಖㆤ⒪ἲࡢ㛤Ⓨࠚ◊✲ࡢᴫせ
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࡟ඛ㥑ࡅ࡚Ⓨ⾲ࡋࡓ $QQDOVRI1HXURORJ\ ࠋࡉࡽ࡟06ࡢ᪂つ἞⒪⸆ࣇ࢕ࣥࢦࣜࣔࢻ࡟
ࡼࡿ㧊⭍ෆච␿⣽⬊ືែࢆྍど໬ࡋࠊ᭹⏝᪩ᮇ࡟࠾ࡅࡿ06෌ⓎࡢࣜࢫࢡᅉᏊࢆゎᯒࡋࡓ 0XOWLSOH
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PXOWLSOH

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

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 ᶓ㛵᫂ᏊἙෆἨす⃝ṇ㇏㐃㍕ ⸆๣ᖌࡀ▱ࡗ࡚࠾ࡃ࡭ࡁ⮚ჾู⏬ീゎᯒࡢᇶ♏▱㆑ ⚄ ⤒ ෆ ⛉ Ꮫ ศ 㔝    ୰ ᯡ ⚄ ⤒ ⣔ ࡢ ⅖ ⑕ ᛶ ⫗ ⱆ ⭘ ᛶ ⑌ ᝈ ࡢ ⏬ ീ デ ᩿  ་ ⸆ ࢪ ࣕ ࣮ ࢼ ࣝ   ✑ⱉ୓ᮤᏊἙෆἨす⃝ṇ㇏㐃㍕ ⸆๣ᖌࡀ▱ࡗ࡚࠾ࡃ࡭ࡁ⮚ჾู⏬ീゎᯒࡢᇶ♏▱㆑  ⚄⤒ෆ⛉Ꮫศ㔝  ୰ᯡ⚄⤒⣔࡟࠾ࡅࡿ⮬ᕫච␿ᛶ⑌ᝈࡢ⏬ീデ᩿ ་⸆ࢪ࣮ࣕࢼࣝ
   ἙෆἨす⃝ṇ㇏≉㞟㸸ከⓎᛶ◳໬⑕࡜ど⚄⤒⬨㧊⅖,,,᳨ᰝ࣭デ᩿ἲ⚄⤒ᨺᑕ⥺Ꮫⓗ᳨ᰝ 05, ᪥ᮏ⮫ᗋ   ἙෆἨす⃝ṇ㇏≉㞟㸸ከⓎᛶ◳໬⑕ࡢࣃࣛࢲ࢖࣒ࢩࣇࢺ̽⑓ែ࠿ࡽ἞⒪ࡲ࡛%ྛㄽ
᳨ᰝI⚄⤒ᚰ⌮᳨ᰝ&OLQLFDO1HXURVFLHQFH   ἙෆἨす⃝ṇ㇏≉㞟㸸⮬ᕫච␿ᛶ⬻⅖㸸ᢠཎ࣭ᢠయࡣఱࢆࡋ࡚ࡿ")࢔ࢡ࢔࣏ࣜࣥ࢔ࢡ
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࠮ࡦ࠲࡯㐳ᢎ᝼ ੖ච፲ ඳਛ ↢૕⏛᳇౒㡆ቇಽ㊁ᢎ᝼ ᩑ↰ ᣿⟤࠺ࠫ࠲࡞කቇಽ㊁ಎᢎ᝼ 

᧻Ỉ ╬

⣖ᯏ⢻⸃ᨆቇಽ㊁ಎᢎ᝼ ㋈ᧁ ᷡ㓉↢૕⏛᳇౒㡆ቇಽ㊁

ಎᢎ᝼ ㋈ᧁ 㓶ᴦ

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⥃ᐥᯏ⢻⣖␹⚻⑼ቇಽ㊁ഥᢎ દ⮮ ᶈ੺

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↢૕⏛᳇౒㡆ቇಽ㊁ഥᢎ ਛ᧛ ੧ᒎ

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⥃ᐥᯏ⢻⣖␹⚻⑼ቇಽ㊁ഥᢎ ർᶆ ᒄ᮸

⥃ᐥᯏ⢻⣖␹⚻⑼ቇಽ㊁ഥᢎ ᬀᧁ ᥓᔒ

⥃ᐥᯏ⢻⣖␹⚻⑼ቇಽ㊁ ⿥ၞቇⴚ㒮 ഥᢎ ୖㇱ ⡡

⥃ᐥᯏ⢻⣖␹⚻⑼ቇಽ㊁ታ㛎ഥᚻᄢḊ ⹞଻ታ㛎ഥᚻਃ౷ ᣿ᄦ

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+ICTCUJK*5W\WMK;*WDGT8,+FC/0CMCFC60CEGV[NCURCTVCVG&GETGCUGKP#EWVG5VCIGQH
+UEJGOKE5VTQMG#2GTURGEVKXGHTQO'ZRGTKOGPVCNCPF%NKPKECN5VWFKGU/CIP4GUQP/GF5EK'RWD
&GE+ICTCUJK*5W\WMK;-YGG+.0CMCFC69CVGTKPHNWZKPVQEGTGDTQURKPCNHNWKFKUUKIPKHKECPVN[TGFWEGFKPUGPKNGRNCSWGDGCTKPIVTCPUIGPKEOKEGUWRRQTVKPIDGVCCO[NQKF
ENGCTCPEGJ[RQVJGUKUQH#N\JGKOGT UFKUGCUG0GWTQN4GU&GE  



5W\WMK-+ICTCUJK**WDGT8,-KVCWTC*-YGG+.0CMCFC6.KICPFDCUGFOQNGEWNCT/4+
,,8%2'CO[NQKFKOCIKPIKPVTCPUIGPKEOKEG,0GWTQKOCIKPI0QX&GE  

0CVUWOGFC/+ICTCUJK*0QOWTC61IWTC46UWMCOQVQ;-QDC[CUJK6#QMK*1MCOQVQ--CMKVC#6CMCJCUJK*0CMCFC6(WLKK;#EEWOWNCVKQPQHJ[FTQZ[INWVCTCVGKPINKQOCUEQTTGNCVGUYKVJUWTXKXCNCUVWF[D[VGUNCOCIPGVKETGUQPCPEGURGEVTQUEQR[#EVC
0GWTQRCVJQN%QOOWP0QX  



0CMCFC68KTEJQY4QDKPURCEGCPFCSWCRQTKPPGYKPUKIJVUQPCPQNFHTKGPF %TQCV/GF,#WI  



1VUW;/CTECIIK2(GNV\#+UQRG2-QNNQ/0WUUGT</CVJKGW$-CPQ/6UWLKVC/
5CMKOWTC-&KGWFQPPª5#EVKXKV[&GRGPFGPV)CVKPIQH%CNEKWO5RKMGUD[#V[RG- %JCPPGNU%QPVTQNU%NKODKPI(KDGT5KIPCNKPIKP2WTMKPLG%GNN&GPFTKVGU0GWTQP1EV  



0CMCLKOC07GFC/+ICTCUJK*-CVC[COC;6TCPUKGPVFKUCRRGCTCPEGQHYJKVGOCVVGTNGUKQPUQP
EQTTGEVKQPQHJ[RQPCVTGOKC0GWTQN%NKP2TCEV,WPG-CYCOWTC-6CMCJCUJK6-CPC\CYC/+ICTCUJK*0CMCFC60KUJK\CYC/5JKOQJCVC6'HHGEVUQHCPIKQRQKGVKPQPJGOQTTJCIKEVTCPUHQTOCVKQPCPFEGTGDTCNGFGOCCHVGTVKUUWGRNCUOKPQIGPCEVKXCVQTVTGCVOGPVHQTKUEJGOKEUVTQMGKPTCVU2.Q51PG,WP  G



-WTCDG 5 +VQJ - /CVUW\CYC * 0CMCFC 6 (WLKK ; 'ZRCPUKQP QH UGPUQTKOQVQT EQTVKECNCEVKXCVKQPHQTWPKNCVGTCNJCPFOQVKQPFWTKPIEQPVTCNCVGTCNJCPFFGCHHGTGPVCVKQP0GWTQTGRQTV
#RT  

 +ICTCUJK*6UWLKVC/-YGG+.0CMCFC69CVGTKPHNWZKPVQEGTGDTQURKPCNHNWKFKURTKOCTKN[EQPVTQNNGF D[ CSWCRQTKP PQV D[ CSWCRQTKP 1 ,,8%2' /4+ UVWF[ KP MPQEMQWV OKEG
0GWTQTGRQTV,CP   ᬀᧁᥓᔒⷞ␹⚻Ἳߣᄙ⊒ᕈ⎬ൻ∝⌒⑼ ᬀᧁᥓᔒ᛫ࠕࠢࠕࡐ࡝ࡦ  ᛫૕㓁ᕈⷞ␹⚻Ἳᢝፉᢘᖗ ✬ ␹⚻⌒⑼⸻ᢿࠢࡠ࡯࠭ࠕ࠶ࡊࡔ
ࠫࠞ࡞ࡆࡘ࡯␠᧲੩ ᬀᧁᥓᔒⷞ␹⚻Ἳߩᴦ≮ᣇ㊎ߪᦨㄭᄌࠊߞߚߩߢߔ߆޽ߚࠄߒ޿⌒⑼  ⥃Ⴧ  ጟᧄᶈ৻㇢‫ޔ‬㊁᧛ବᤐ‫ୖޔ‬ㇱ⡡‫ޔ‬Ⴆᧄ૫ᐢ‫ޔ‬᫙↰ቇ‫ޔ‬ዊୖ⦟੺‫ޔ‬੖චᎹℰⓄ‫ޔ‬㕍ᧁᵗ‫ޔ‬㊄ᴛീ‫ޔ‬᷆〝ᱜೣ‫ޔ‬ⒷᎹᱜ৻‫ޔ‬੖ච፲ඳਛ‫⮮ޔ‬੗ᐘᒾ ࡑ࡞࠴ࡕ࠳࡝࠹ࠖߦࠃࠆ *GCF0GEM+OCIKPIᦨᣂ
ᛛⴚ߇⥃ᐥ㨚߽ߚࠄߔᄌ㕟ߣࡌࡀࡈࠖ࠶࠻⥃ᐥ✬+/4+ ߩࠬ࠻࡜࠹ࠫ࡯ࠕ࠙࠻ࠞࡓ⥃ᐥᣉ⸳߆ࠄߩႎ๔⣖⣲≌̄ ␹⚻⤔⣲ߩ⸻ᢿ࡮㐓೎⸻ᢿ‫ⴚޔ‬೨ᖱႎขᓧߦ߅ߌࠆ /4+ ߩ᦭↪ᕈࠗࡦ࠽࡯ࡆ࡚ࠫࡦ  ᐕ  ᦬ภ ጊ㊁੗ℂᕺ‫ޔ‬ጟᧄᶈ৻㇢‫ޔ‬ᴡ᧛ ๺♿‫ޔ‬ᄢਭ଻⌀᮸‫ޔ‬㋈ᧁ㓶ᴦ‫ޔ‬ਛ᧛੧ᒎ =1?*115'/ᴺ 2'6↹௝ߢߩ UWDUGVߣ KVGTCVKQPߩ⚵ߺวࠊߖߦࠃࠆ↹⚛୯ߩᬌ⸛㧦($2ᴺࠍၮḰߣߒߡ ᩭකቇ㧧  
㸫㸫Φ ౒ห⎇ⓥ
ਛᩭ⊛⎇ⓥ᜚ὐ㧔%1'㧕ᒻᚑࡊࡠࠣ࡜ࡓ‫ޔ‬ㅪ៤Ⲣว੐ᬺ‫ޟ‬᳓ಽሶߩ⣖⑼ቇ‫․ߦࠄߐࠄ߆ޠ‬೎⚻⾌‫ޟ‬ᗧ⼂ߩ⣖⑼ቇ‫ߡߒߣ࠻ࠢࠚࠫࡠࡊޠ‬ᒁ߈⛮߇ࠇ‫ߩ࠻ࡅޔ‬ᗧ⼂ߣᔃࠍมࠆ⣖ᯏ᭴ߩ⋥ធ⸃᣿ࠍታ〣ߔࠆ⎇ⓥ࡮ᢎ⢒ߩ࿖㓙⊛᜚ὐᒻᚑࠍផㅴߒߡ޿ࠆ‫ޕ‬



⎇ⓥ㗴⋡

․೎⚻⾌㧔ࡊࡠࠫࠚࠢ࠻ಽ㧕‫ޟ‬ᗧ⼂ߩ⣖⑼ቇ‫ޠ‬㧔ᐔᚑᐕ㨪ᐕᐲ㧕࿖㓙⊛ߦථ⿧ߒߚᢎ⢒⎇ⓥ᜚ὐᯏ⢻ߩలታ⎇ⓥౝኈ⿥㜞⸃௝ᐲ᳓ಽሶ↹௝ߣࠕࠢࠕࡐ࡝ࡦ㧙㧠ಽሶ↹௝ߣࠍ㚟૶ߒߚ࿖㓙⎇ⓥ⚵     ❱ߩ᦭ᯏ⊛⛔วߦࠃࠅ‫ޔ‬᳓ಽሶߩേᘒ⸃ᨆࠍㅢߒߡࡅ࠻ߩᗧ⼂ࠍมࠆ⣖ߩ⛔    วᯏ᭴ߩ⸃᣿ࠍ⋡ᜰߔ‫ޕ‬ෳടᯏ㑐

0GWTQNQI[7PKXGTUKV[QH%CNKHQTPKC&CXKU

☨࿖ 

⎇ⓥ㗴⋡

%#32ಽሶ↹௝ᴺߩ⥃ᐥ⎇ⓥ㧔ᐔᚑᐕ㨪ᐕᐲ㧕⎇ⓥౝኈ ᒰᣉ⸳ߢ㐿⊒ߒߚ#32࡝ࠟࡦ࠼ߩ⥃ᐥᔕ↪ࠍ⋡ᜰߔ‫ޕ‬ෳടᯏ㑐ࠟ࠴࡚ࡦක⑼ᄢቇ

ᄢ㖧᳃࿖ 

㸫㸫ㆮવሶᯏ⢻⸃ᨆቇಽ㊁࡮↢๮ᖱႎᎿቇಽ㊁Σ ⎇ⓥ⚵❱㧔᭴ᚑຬ ᐔᚑᐕ᦬ᣣ⃻࿷㧕ᢎ ᝼    ᳰౝ ஜഥ ᢎ

ᤐᣣஜ૞㧔⿥ၞቇⴚ㒮㧕           ഥ ᢎ    ችਅືౖ㧔ભ⡯ਛ㧕⎇ⓥຬ 


⩵࿾ᱜ㓉



᦬ᳯ⃨✜

ᛛⴚ⡯ຬ

㣐⷗ౖሶ





ᷓᶏ᥍ઍ





ฎᎹ⧷ℂ





ේ ▸๺





૒⮮ᕀᄹ





⷗↰㗅ሶ





ᴡว㤀ሶ

੐ോ⡯ຬ

㜞Ლᕺሶ


Τ ⎇ⓥᵴേ
㧔㧝㧕ో࿖ߩ⹺⍮∝㑐ㅪ∔ᖚ࡮ㆮવሶ⸃ᨆ࠮ࡦ࠲࡯ߣߒߡᵴേߒߡ޿ࠆ‫ߟߦ∛࡯ࡑࠗࡂ࠷࡞ࠕߦ․ޕ‬޿ߡߪ࿖ౝᦨᄢߩࠥࡁࡓDNAࠍ㓸Ⓧߒߡ޿ࠆ‫ߚ߹ޕ‬㧘ኅᣖᕈࠕ࡞࠷ࡂࠗࡑ࡯∛ߩㆮવሶ⸃ᨆࠍ
ో࿖ߩฦᣉ⸳߆ࠄଐ㗬ࠍฃߌ㧘ᡰេࠍⴕߞߡ޿ࠆ‫ޕ‬㧔㧞㧕౒ห⎇ⓥߣߒߡ2-1) ࿖㓙౒ห⎇ⓥDIAN-Japanࡊࡠࠫࠚࠢ࠻ߩ↢ൻቇ࡮ㆮવሶ⸃ᨆࠦࠕᵴേ
2-2) ࿖ౝᄙᣉ⸳౒ห⎇ⓥ JALPAC ⎇ⓥߩ↢૕⹜ᢱࡃࡦࠢᵴേ
2-3) ૒ᷰፉߩፉ᳃ࠍኻ⽎ߦߒߚ⥃ᐥ∉ቇ⎇ⓥ‫ޟ‬PROST㧔Project in SADO for Total Health㧕‫ޠ‬ߦ៤ࠊߞߡ޿ࠆ‫ߢ࠻ࠢࠚࠫࡠࡊߩࠄࠇߎޕ‬෼㓸ߒߚࡅ࠻࡝࠰࡯ࠬ㧔ⴊᶧ‫ⴊޔ߁ࠂߒⴊޔ‬ᷡ‫ޔ‬⣖⣄㜑ᶧ‫ޔ‬ዩ‫ޔ‬ਇᱫൻ࡝ࡦࡄ⃿‫ޔ‬ᱫᓟ⣖⚵❱‫ࡓࡁࠥޔ‬DNA‫ޔ‬RNA╬㧕ࠍ▤ℂ࡮଻▤ߒߡ޿ࠆ‫ޕ‬㧔㧟㧕ో࿖౒ห೑↪࡮౒ห⎇ⓥߩផㅴߦ‫ޔ‬ᰴ਎ઍࠪ࡯ࠢࠛࡦࠨ࡯ࠍਛᔃߣߒߚᄢဳ⸳஻ߩᵴ↪෸߮ᛛⴚࠍឭଏߒߡ޿ࠆ‫ޕ‬㧔㧠㧕ᒰಽ㊁ߩ୘೎⎇ⓥߣߒߡ4-1)ࠕ࡞࠷ࡂࠗࡑ࡯∛ߩࠥࡁࡓ⸃ᨆ
4-2)ࡅ࠻ಓ⚿⣖ࠍ↪޿ߚࠕ࡞࠷ࡂࠗࡑ࡯∛ߩ∛൓ࠍ෻ᤋߔࠆㆮવሶߩ✂⟜⊛⸃ᨆ⎇ⓥ
4-3)↢૕⹜ᢱࠍ↪޿ߚࠕ࡞࠷ࡂࠗࡑ࡯∛ߩࡃࠗࠝࡑ࡯ࠞ࡯⸃ᨆ
ࠍⴕߞߡ޿ࠆ‫ޕ‬Υ ⺰ᢥ㧔ේ⪺‫଀∝ޔ⺑✚ޔ‬ႎ๔ࠍ඙೎ߒߥ޿㧕
1.Tokutake T, Ishikawa A, Yoshimura N, Miyashita A, Kuwano R, Nishizawa M, Ikeuchi T. Clinical andneuroimaging features of patient with early-onset Parkinson's disease with dementia carrying SNCA
p.G51D mutation. Parkinsonism & Related Disord 20:262-264, 2014
2. Kasuga K, Konno T, Saito K, Ishihara A, Nishizawa M, Ikeuchi T. A Japanese family of idiopathic
basal ganglia calcification with novel SLC20A2 mutation presenting late-onset hallucination anddelusion. Journal of Neurology 261:242-244, 2014㸫㸫

3. Konno T, Tada M, Tada M, Koyama A, Nozaki H, Harigaya Y, Nishimiya J, Matsunaga A, Yoshikura N,
Ishihara K, Arakawa M, Isami A, Okazaki K, Yokoo H, Itoh K, Yoneda M, Kawamura M, Inuzuka T,
Takahashi H, Nishizawa M, Onodera O, Kakita A, Ikeuchi T. Haploinsufficiency of CSF-1R andClinicopathological Characterization in patients with HDLS. Neurology 82:139-148, 2014
4. Tagami S, Okochi M, Yanagida K, Kodama T, Arai T, Kuwano R, Ikeuchi T, Takeda M. Relative Ratioand Level of Amyloid-ȕ 42 Surrogate in Cerebrospinal Fluid of Familial Alzheimer Disease Patientswith Presenilin 1 Mutations. Neurodegener Disease 13:166-70, 2014
5. Miyashita A, Hatsuta H, Kikuchi M, Nakaya A, Saito Y, Tsukie T, Hara K, Ogishima S, Kakita A,
Takahashi H, Murayama S, Ihara Y, Ikeuchi T, Kuwano R. Genes associated with the progression ofneurofibrillary tangles in Alzheimer’s disease brain. Translational Psychiatry 4, e396, 2014
6. Miyashita A, Wen Y, Kitamura N, Matsubara E, Kawarabayashi T, Shoji M, Tomita N, Arai H, Asada T,
Harigaya Y, Ikeda M, Amari M, Hanyu H, Higuchi S, Nishizawa M, Suga M, Kawase Y, Akatsu H,
Imagawa M, Hamaguchi T, Yamada M, Morihara T, Takeda M, Takao T, Nakata K, Sasaki K,
Watanabe K, Nakajima K, Urakami K, Ooya T, Takahashi M, Yuzuriha T, Serikawa K, Yoshimoto S,
Nakagawa R, Saito Y, Hatsuta H, Murayama S, Kakita A, Takahashi H, Yamaguchi H, Akazawa K,
Kanazawa I, Ihara Y, Ikeuchi T, Kuwano R. Lack of genetic association between TREM2 and lateonset Alzheimer’s disease in the Japanese population. Journal of Alzheimer’s disease 41:10311038, 2014Φ ౒ห⎇ⓥ(1) ⎇ⓥ㗴⋡㧦‫ޟ‬ኅᣖᕈࠕ࡞࠷ࡂࠗࡑ࡯∛ߦ㑐ߔࠆ❑ᢿ⊛ⷰኤࠦࡎ࡯࠻⎇ⓥ‫ޠ‬⎇ⓥౝኈ㧦ේ࿃ߣߥࠆㆮવሶᄌ⇣߇หቯߐࠇߚኅᣖᕈࠕ࡞࠷ࡂࠗࡑ࡯∛ߩኅ♽ຬࠍኻ⽎ߣߒߚ❑ᢿ⊛ࠦࡎ࡯࠻⎇ⓥߢ޽ࠆ‫⹺ޕ‬⍮∝ࠍ⊒∝ߔࠆߦ⥋ࠆࡃࠗࠝࡑ࡯ࠞ࡯ߩᄌൻࠍ᣿ࠄ߆ߦ
ߔࠆࡊࡠࠫࠚࠢ࠻ߢ޽ࠆ‫ޕ‬ෳടᯏ㑐㧦ᄢ㒋Ꮢ┙ᄢቇ‫ޔ‬ᒄ೨ᄢቇ㧘᧲੩ᄢቇ㧘᧲੩ㇺஜᐽ㐳ኼක≮࠮ࡦ࠲࡯‫ޔ‬వ┵ක≮࠮ࡦ࠲࡯㧘᧲ർᄢቇ㧘ᄢಽᄢቇ㧘ጊ᪸ᄢቇ㧘ᾢᧄᄢቇ(2) ⎇ⓥ㗴⋡㧦‫⹺ޟ‬⍮∝ߩᩮᧄ⊛ߥේ࿃ߩ⸃᣿ࠍ⋡ᜰߒߚࠦࡎ࡯࠻⎇ⓥߣ✂⟜⊛ࠥࡁࡓ㈩೉⸃ᨆ⎇ⓥ‫ޠ‬⎇ⓥౝኈ㧦ࠕ࡞࠷ࡂࠗࡑ࡯∛ߩ⊒∝ߦ㑐ਈߔࠆㆮવ⊛࡝ࠬࠢࠍ᣿ࠄ߆ߦߔࠆ⋡⊛ߢ❑ᢿ⊛ࠦࡎ࡯࠻⎇ⓥࠍⴕ߁ߣ౒ߦ㧘ኅᣖᕈࠕ࡞࠷ࡂࠗࡑ࡯∛ห⢩⊒∝଀ߩㆮવሶ⸃ᨆߦࠃࠅ࡟ࠕࡃ࡝ࠕ
ࡦ࠻ߦࠃࠆ࡝ࠬࠢᄙဳߩหቯࠍ⹜ߺࠆ‫ޕ‬ෳടᯏ㑐㧦᧲੩ᄢቇ
(3) ⎇ⓥ㗴⋡㧦‫⚻␹ޟ‬ゲ⚝ࠬࡈࠚࡠࠗ࠼ᒻᚑࠍ઻߁ㆮવᕈ߮߹ࠎᕈ⊕⾰⣖∝㧔HDLS)ߩ⸻ᢿၮḰߩ╷ቯ‫ޠ‬⎇ⓥౝኈ㧦␹⚻ゲ⚝ࠬࡈࠚࡠࠗ࠼ᒻᚑࠍ઻߁ㆮવᕈ߮߹ࠎᕈ⊕⾰⣖∝㧔HDLS㧕ߦߟ޿ߡㆮવሶ⸻
ᢿߢ⏕ቯ⸻ᢿߐࠇߚ∝଀ࠍ↪޿㧘⥃ᐥ࡮↹௝⊛ߥ․ᓽࠍ᣿ࠄ߆ߦߔࠆߣߣ߽ߦ㧘⸻ᢿၮḰࠍ╷ቯߔࠆ‫ޕ‬ෳടᯏ㑐㧦ାᎺᄢቇ‫ޔ‬੩ㇺᐭ┙ක⑼ᄢቇ‫ޔ‬ᓼፉᄢቇ㸫㸫ື≀㈨※㛤Ⓨ◊✲ศ㔝Ϩ ◊✲⤌⧊㸦ᵓᡂဨ ᖹᡂᖺ᭶᪥⌧ᅾ㸧ᩍᤵ     ➲ᒸ ಇ㑥ຓᩍ     ⸨⃝ ಙ⩏ຓᩍ     ๓⏣ ᐅಇ≉௵ຓᩍ   ᑠ⏣ ెዉᏊࣇ࢙࣮ࣟ   ᶓᒣ ᓟ௓㠀ᖖ໅ㅮᖌ  బ⸨ ಇဢ ᢏ⾡ᑓ㛛⫋ဨ ⏣୰ ⛱஦ົ⫋ဨ   ຍ⸨ ᫂Ꮚᢏ⾡⫋ဨ   㑣㡲㔝 ⣧ᫎ஦ົ⿵బဨ  ⏣௦ ᬛᏊ஦ົ⿵బဨ  ஂఫ ┿⏤⨾ὴ㐵ᢏ⾡⫋ဨ ᒣᮏ ⨾ୣὴ㐵ᢏ⾡⫋ဨ 㓇஭ ΎᏊὴ㐵ᢏ⾡⫋ဨ ⚄ಖ ᖾᘯὴ㐵ᢏ⾡⫋ဨ ༓ⴥ ࡉ࠾ࡾὴ㐵ᢏ⾡⫋ဨ 㤿ᕝ ᜨ᲍Ꮚϩ ◊✲άື ࣃ࣮࢟ࣥࢯࣥ⑓ࡢ㐠ື㞀ᐖࡢᶵᵓゎ᫂ࡢࡓࡵࠊ'ࢻ࣮ࣃ࣑ࣥཷᐜయ '5 ࢆ௓ࡍࡿࠕ┤᥋㊰ࠖ࠾
ࡼࡧ'ࢻ࣮ࣃ࣑ࣥཷᐜయ '5 ࢆ௓ࡍࡿࠕ㛫᥋㊰ࠖ࡟╔┠ࡋࠊ'5࣭'5ࢆ⤒⏤ࡍࡿ᝟ሗࡢ㐠ືไᚚࡢᙺ๭ࡢゎ᫂◊✲ࢆ㐍ࡵ࡚࠸ࡿࠋ'5ࢥࣥࢹ࢕ࢩࣙࢼࣝ㑇ఏᏊⓎ⌧࣐࢘ࢫࢆ⏝࠸࡚'5ࡢࡳࢆⓎ⌧Ḟᦆࡉࡏࡿᐇ㦂⣔࡛ࠊ'5ࢆ௓ࡍࡿࠕ┤᥋㊰ࠖࡀ㐠ື㔞ࢆ⥔ᣢࡋ㐠ືࢆಁ㐍ࡍࡿࡇ࡜ࢆぢฟࡋࡓࠋࡉࡽ࡟
'5ࡢᙺ๭࡟ࡘ࠸࡚ࠊ⥺᮲యࡢࢥࣜࣥసືᛶ௓ᅾ⚄⤒⣽⬊࡟࠾࠸࡚'5ࡀ*$%$ࡢᨺฟࢆไᚚࡋ࡚࠸ࡿࡇ࡜ࢆぢฟࡋࡓࠋ
 ࣐࢖ࢡࣟ࢖ࣥࢪ࢙ࢡࢩࣙࣥἲ࡟ࡼࡿᶆⓗ㑇ఏᏊᨵኚ࣐࢘ࢫࡢసᡂ࡟ᣮᡓࡋ࡚࠸ࡿࠋேᕤไ㝈㓝⣲ᢏ⾡ࡢඛ㥑ࡅ࡜࡞ࡗࡓ=LQF)LQJHU1XFOHDVH㸦=)1㸧ᢏ⾡ࢆᖺ࡟ᑟධࠊࡉࡽ࡟➨୕ୡ௦ࡢᨵኚ
ᢏ⾡࡛࠶ࡿ&5,635&DVࢩࢫࢸ࣒ࡢᑟධ࡟ࡶᡂຌࡋࡓࠋᮏᢏ⾡ࡣࠊ㑇ఏᏊᨵኚື≀సᡂࡢࣁ࣮ࢻࣝࢆ
୍Ẽ࡟ୗࡆࠊ௒ᚋࡢ◊✲Ⓨᒎ࡟ᚲ㡲࡛࠶ࡿࡓࡵࠊᙜศ㔝࡛ࡶ⮬ᅾ࡟ᢅ࠼ࡿែໃࢆᩚ࠼࡚࠸ࡿࠋ ⏕ṪᕤᏛⓗᢏ⾡ࢆ⏝࠸ࠊࢻ࣮ࣃ࣑ࣥ≉␗ཷᐜయḞᦆ࣐࢘ࢫ➼ࡢ⬻࣭⚄⤒⣔⑌ᝈࡢ⑓ែࣔࢹࣝࢆ
ࡣࡌࡵࠊྛ✀㑇ఏᏊᨵኚ࣐࢘ࢫࢆ౪⤥ࡋ࡚࠸ࡿࡀࠊࡇࡢᢏ⾡ࢆ⏝࠸࡚⏕ࡲࢀࡓ࣐࢘ࢫࡣࠊయ㔜ࡀቑຍࡍࡿഴྥ࡟࠶ࡿࡇ࡜ࢆぢ࠸ࡔࡋࡓࠋࡑࡇ࡛⏕ṪᕤᏛⓗ᧯స࡛ᚲ㡲࡛࠶ࡿయእᇵ㣴ࡀึᮇ⬇ࡢⓎ⏕࠾ࡼࡧಶయⓎ⏕࡟ᙳ㡪ࢆཬࡰࡍ࠿᫂☜࡟ࡍࡿᚲせࡀ࠶ࡾ◊✲ࢆ⾜ࡗ࡚࠸ࡿࠋࡇࢀࡲ࡛࡟యእᇵ㣴⬇
ࡢ࢖ࣥࣉࣜࣥࢺ㑇ఏᏊ࡟Ⓨ⌧ኚ໬ࡀ࠶ࡿࡇ࡜ࡀศ࠿ࡗࡓࠋ  ື≀ᐇ㦂᪋タࡢ⪁ᮙ໬࡜⪏㟈ᛶᙉ໬ࡢࡓࡵࠊᖹᡂᖺ࡟኱つᶍᨵಟᕤ஦ࢆ⾜࠸ࠊ௬᪋タ࡛ࡢᐇ㦂⥅⥆࠿ࡽᨵಟ᪋タ࡬ࡢ⛣㌿ࠊ෌❧ࡕୖࡆࢆ⤒࡚ࠊᖹᡂᖺࡼࡾᮏ᱁✌ാࢆ㛤ጞࡋࡓࠋࡑࡢᚋࠊᶆ
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313/$㑇ఏᏊࡢ⬻࡟࠾ࡅࡿᶵ⬟᭷ᶵࣜࣥ⿕⇿࡜ࡢ㛵㐃࠿ࡽ◊✲௦⾲⪅㸸ᮌᮧ✨ᩍᤵ㸦ᮾᾏ኱Ꮫ㸧ᖹᡂᖺᗘ ඹྠ฼⏝࣭ඹྠ◊✲㸦ࣉࣟࢪ࢙ࢡࢺᆺ㸧ࢻ࣮ࣃ࣑ࣥཷᐜయࣀࢵࢡ࢔࢘ࢺ࣐࢘ࢫࢆ⏝࠸ࡓ୰ᯡࢩࢼࣉࢫఏ㐩ᶵᵓࡢゎᯒ◊✲௦⾲⪅㸸⢄ᒣಇᙪᩍᤵ㸦ᮾிឿᜨ఍་⛉኱Ꮫ㸧ᖹᡂᖺᗘ ඹྠ฼⏝࣭ඹྠ◊✲㸦ࣉࣟࢪ࢙ࢡࢺᆺ㸧
%4/1ࢥࣥࢹ࢕ࢩࣙࢼࣝࣀࢵࢡ࢔࢘ࢺ࣐࢘ࢫࡢゎᯒ࡟ᇶ࡙ࡃ⚄⤒ኚᛶᶵᗎࡢゎ᫂◊✲௦⾲⪅㸸⏣୰❶ᬒᩍᤵ㸦ᶓ὾ᕷ❧኱Ꮫ㸧ᖹᡂᖺᗘ ඹྠ฼⏝࣭ඹྠ◊✲㸦ࣉࣟࢪ࢙ࢡࢺᆺ㸧့ங㢮୰ᯡ⚄⤒⣔࡟࠾ࡅࡿ⚄⤒ᅇ㊰ᙧᡂࡢ㑇ఏᏛⓗゎᯒ◊✲௦⾲⪅㸸ᒾ㔛⌶἞ᩍᤵ㸦ᅜ❧㑇ఏᏛ◊✲ᡤ㸧 ᖹᡂᖺᗘ ඹྠ฼⏝࣭ඹྠ◊✲㸦ࣉࣟࢪ࢙ࢡࢺᆺ㸧኱⬻ᇶᗏ᰾ෆ᝟ሗఏ㐩࡟࠾ࡅࡿࢻ࣮ࣃ࣑ࣥ⚄⤒ఏ㐩ࡢᶵ⬟ࡢゎᯒ◊✲௦⾲⪅㸸༡㒊⠜ᩍᤵ㸦⮬↛⛉Ꮫ◊✲ᶵᵓ⏕⌮Ꮫ◊✲ᡤ㸧 ᖹᡂᖺᗘ ඹྠ฼⏝࣭ඹྠ◊✲㸦㐃ᦠ㈨※฼⏝ᆺ㸧ࢻ࣮ࣃ࣑ࣥཷᐜయኚ␗࣐࢘ࢫࢆ⏝࠸ࡓ୙Ᏻᵝ⾜ືⓎ⑕ᶵᗎࡢゎ᫂◊✲௦⾲⪅㸸㧗ᶫṇ㌟ᩍᤵ㸦໭㔛኱Ꮫ㸧 ᖹᡂᖺᗘ ඹྠ฼⏝࣭ඹྠ◊✲㸦㐃ᦠ㈨※฼⏝ᆺ㸧⫾௘ᮇ࠾ࡼࡧⓎ㐩ᮇࡢ⬻࡟࠾ࡅࡿࢻ࣮ࣃ࣑ࣥཷᐜయ'5ࡢᶵ⬟ゎᯒ◊✲௦⾲⪅㸸኱ஂಖ┤෸ᩍᤵ㸦໭㔛኱Ꮫ㸧㸫㸫 ᖹᡂᖺᗘ ඹྠ฼⏝࣭ඹྠ◊✲㸦㐃ᦠ㈨※฼⏝ᆺ㸧
$33⣽⬊ෆࢻ࣓࢖ࣥࡢ⚄⤒ẘᛶࡢゎᯒ◊✲௦⾲⪅㸸୰ᒣ⪔㐀ㅮᖌ㸦ಙᕞ኱Ꮫ㸧 ᖹᡂᖺᗘ ඹྠ฼⏝࣭ඹྠ◊✲㸦㐃ᦠ㈨※฼⏝ᆺ㸧ࢻࣃ࣑ࣥ'5ࢩࢢࢼࣝࡀᚰ୙඲࡟ᯝࡓࡍᙺ๭ࡢゎ᫂◊✲௦⾲⪅㸸ᑠᐊ୍ᡂᩍᤵ㸦ᮾி኱Ꮫ㸧㸫㸫ಽሶ␹⚻∔ᖚ⾗Ḯ⸃ᨆቇಽ㊁ Σ ⎇ⓥ⚵❱㧔᭴ᚑຬ ᐔᚑᐕ᦬ᣣ⃻࿷㧕   ᢎ᝼   ዊ㊁ኹ ℂ   ഥᢎ   ⍹ේᥓᒾ
ᛛⴚ⡯ຬ ᑝᎹ␽ሶΤ ⎇ⓥᵴേ
ᧄᢎቶߪ␹⚻∔ᖚߩಽሶ↢‛ቇ⊛⸃ᨆߦࠃࠅ㧘∛ᘒᯏᐨࠍ᣿ࠄ߆ߦߒ㧘ᦨ⚳⊛ߦߪ␹⚻∔ᖚߩ᦭ലߥᴦ≮ᣇᴺߩ㐿⊒ࠍⴕ߁ߎߣ⋡⊛ߣߒߡ޿ࠆ㧚ᧄቇ⣖⎇ⓥᚲ㧘␹⚻ౝ⑼ቇᢎቶߩᡰេࠍฃߌ
ߡ㧘⥃ᐥߣߩⲢว᜚ὐߣߒߡᵴേࠍផㅴߒߡ޿ࠆ㧚߹ߚ∛ℂቇᢎቶ㧘േ‛ታ㛎ᣉ⸳㧘ㆮવሶታ㛎ᣉ⸳ࠍਛᔃߣߔࠆ㧘⣖⎇ⓥᚲߩฦᢎቶ㧘߅ࠃ߮࿖ౝ㧘࿖ᄖߩ⎇ⓥቶߣ߽౒ห⎇ⓥࠍផㅴߒߡ޿ࠆ㧚ᒰᣉ⸳ߢߪ․ߦㆮવᕈ⣖ዊⴊ▤∛㧘╭⪜❗ᕈ஥⚝⎬ൻ∝㧔#.5㧕㧘⣄㜑ዊ⣖ᄌᕈ∝ߩฦ∔ᖚߦߟ޿ߡ⎇ⓥࠍផㅴߒߡ޿ࠆ㧚  ㆮવᕈ⣖ዊⴊ▤∛㧘․ߦEGTGDTCNCWVQUQOCNTGEGUUKXGCTVGTKQRCVJ[YKVJUWDEQTVKECNKPHCTEVUCPF
NGWMQGPEGRJCNQRCVJ[㧔%#4#5+.㧕ߦߟ޿ߡ㧘ಽሶ∛ᘒᯏᐨ߆ࠄߩಽᨆ࡮⸃᣿ࠍⴕߞߡ޿ࠆ㧚ᧄᐕᐲߪ㧘ᣢ
ႎߩ%#4#5+.∝଀ߦߟ޿ߡ㧘⥃ᐥ⊛㧘ಽሶ∛ᘒቇ⊛ߥᬌ⸛ࠍട߃ߚ✚⺑ࠍUVTQMG⹹ߦ⊒⴫ߒߚ㧚ᧄ⹹ߪ⣖තਛಽ㊁ߢᦨ߽ᮭᆭ޽ࠆቇળ⹹ߢ޽ࠆ㧚ᧄቇળ⹹ߩᣣᧄ߆ࠄߩ✚⺑⺰ᢥߪᭂ߼ߡᏗߢ޽ࠅ㧘ㆊ෰ᐕ㑆ߩᧄߩឝタ✚⺑ߩ߁ߜ㧘ᣣᧄ߆ࠄߩ✚⺑ߪᧄ✚⺑ߩߺߢ޽ࠆ㧚ߎࠇߪ࿖㓙⊛ߦᧄ⎇ⓥߩ⹏ଔ߇ᭂ߼ߡ㜞ߊ㧘ᧄ⎇ⓥ㗔ၞߩ╙৻ੱ⠪ߣߒߡ⹺⍮ߐࠇߡ޿ࠆߎߣࠍ␜ߔ߽ߩߢ޽ࠆ㧚
#.5ߦߟ޿ߡߪ㧘6&2O40#ߩዪ࿷࡮⊒⃻㊂⺞▵ߦ㑐ߔࠆ⎇ⓥ㧘ᯏ⢻ᕈ40#ઍ⻢ߦ㑐ㅪߒߚᣂߚߥ∛ᘒᯏᐨߦ㑐ߔࠆ⎇ⓥ㧘ࡒ࠻ࠦࡦ࠼࡝ࠕઍ⻢㑐ㅪⰮ⊕⾰O40#ߦߟ޿ߡߩ⎇ⓥࠍⴕ޿㧘࿖ౝᄖߩቇળߦߡ⊒⴫ࠍⴕߞߡ޿ࠆ㧚߹ߚᐕ᦬ߦߪ㧘ᯏ⢻ᕈ40#ઍ⻢ߦ㑐ㅪߒߚ⎇ⓥߦߟ޿ߡ╙৻࿁ᣂẟᄢቇቇ㐳⾨㧔⧯ᚻᢎຬ⎇ⓥᅑബ㧕ࠍฃ⾨ߒߡ޿ࠆ㧚#.5ߪ6&2㧘(75㧘%QTHߥߤ∔ᖚ㑐ㅪㆮવሶ㧘Ⱞ⊕⾰ߩ⊒⷗ࠍ┵✜ߣߒߡ㧘∛ᘒᯏᐨ⸃᣿ߦะߌߡ࿖㓙⊛ߥ┹੎߇ⴕࠊࠇߡ޿ࠆ㧚ߘߩਛߢᧄᣉ⸳ߢߪ40#ઍ⻢ߦᵈ⋡ߒߡ#.5∛ᘒᯏᐨߩ⸃ᨆࠍⴕߞߡ޿ࠆὐߦ․⦡߇޽ࠆ㧚౒ห⎇ⓥߢߪ㧘ෘ↢ഭ௛⋭⎇ⓥ⾌⵬ഥ㊄ߦࠃࠅ㧘̌6)(Ǫࠪࠣ࠽࡞ߦᵈ⋡ߒߚ%#4#5+.ߩ↹ᦼ⊛ᴦ≮ᣇᴺߩᬌ⸛̍ࠍផㅴߒ㧘ᣂߚߥ%#4#5+.ߩಽሶᮡ⊛≮ᴺࠍ㐿⊒ߒߚ㧚߹ߚ⎇ⓥ⾗㊄ߪ⑼ቇ⎇ⓥ⾌⵬ഥ㊄ $ ࠍᆎ߼ߣߒߡ㧘ฦ‫⎇ߩޘ‬ⓥ⠪߇┹੎⊛⎇ⓥ⾗㊄ࠍ₪ᓧߒ㧘
⎇ⓥᵴേࠍផㅴߒߚ㧚Υ ⺰ᢥ㧔ේ⪺‫଀∝ޔ⺑✚ޔ‬ႎ๔ࠍ඙೎ߒߥ޿㧕㩷
1.

Nozaki H, Nishizawa M, Onodera O. Features of Cerebral Autosomal Recessive Arteriopathy
with Subcortical Infarcts and Leukoencephalopathy. Stoke 2014;45:3447-3453.

2.

Konno T, Tada M, Shiga A, Tsujino A, Eguchi H, Masuda-Suzukake M, Hasegawa M,
Nishizawa M, Onodera O, Kakita A, Takahashi H. C9ORF72 repeat-associated non-ATGtranslated polypeptides are distributed independently of TDP-43 in a Japanese patient with
c9ALS. Neuropathology and applied neurobiology, 2014;40:783-788
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Onodera O, Ishihara T, Shiga A, Ariizumi Y, Yokoseki A, Nishizawa M. Minor splicing
pathway is not minor any more: implications for the pathogenesis of motor neuron diseases.
Neuropathology, 2014;34:99-107

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Konno T, Tada M, Tada M, Koyama A, Nozaki H, Harigaya Y, Nishimiya J, Matsunaga A,
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Kawamura M, Inuzuka T, Takahashi H, Nishizawa M, Onodera O, Kakita A, Ikeuchi T.
Haploinsufficiency of CSF-1R and clinicopathologic characterization in patients with HDLS.
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Yasui K, Yabe I, Yoshida K, Kanai K, Arai K, Ito M, Onodera O, Koyano S, Isozaki E, Sawai S,
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Nakashima K. A 3-year cohort study of the natural history of spinocerebellar ataxia type 6 inJapan. Orphanet journal of rare diseases, 2014;9:118

6.

Kensaku Kasuga, Takuya Konno, Kento Saito, Ayako Ishihara, Masatoyo Nishizawa, Takeshi
Ikeuchi. A Japanese family with idiopathic basal ganglia calcification with novel SLC20A2
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withlate-onset

hallucinationanddelusion.

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Fu YJ, Aida I, Tada M, Tada M, Toyoshima Y, Takeda S, Nakajima T, Naito H, Nishizawa M,
Onodera O, Kakita A, Takahashi H. Progressive myoclonus epilepsy: extraneuronal brown
pigment deposition and system neurodegeneration in the brains of Japanese patients withnovel SCARB2 mutations. Neuropathology and applied neurobiology, 2014;40:551-563

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Kimura T, Jiang H, Konno T, Seto M, Iwanaga K, Tsujihata M, Satoh A, Onodera O, Kakita A,
Takahashi H. Bunina bodies in motor and non-motor neurons revisited: a pathological studyof an ALS patient after long-term survival on a respirator. Neuropathology : official journal of
the Japanese Society of Neuropathology, 2014;34:392-39

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Chizuru Akimoto, Alexander E Volk, Marka van Blitterswijk, Marleen Van den Broeck, Claire
S Leblond, Serge Lumbroso, William Camu, Birgit Neitzel, Osamu Onodera, Wouter van
Rheenen, Susana Pinto, Markus Weber, Bradley Smith, Melanie Proven, Kevin Talbot,
Pamela Keagle, Alessandra Chesi, Antonia Ratti, Julie van der Zee, Helena Alstermark, Anna
Birve, Daniela Calini, Angelica Nordin, Daniela C Tradowsky, Walter Just, Hussein Daoud,Sabrina Angerbauer, Mariely DeJesus-Hernandez, Takuya Konno, Anjali Lloyd-Jani,
Mamede de Carvalho, Kevin Mouzat, John E Landers, Jan H Veldink, Vincenzo Silani, Aaron
D Gitler, Christopher E Shaw, Guy A Rouleau, Leonard H van den Berg, Christine Van
Broeckhoven, Rosa Rademakers, Peter M Andersen, Christian Kubisch. A blindedinternational study on the reliability of genetic testing for GGGGCC-repeat expansions inC9orf72 reveals marked differences in results among 14 laboratories. J Med Genet 2014
Jun;51(6):419-424

10. ਄᧛᣽ኡ, ㊁ፒᵗ᣿㧘 ⷏Ỉᱜ⼾㧘 ዊ㊁ኹℂ㧚 CADASILߣCARASIL. ᣣᧄ⥃ᐥ 2014;72:619623.
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11. 㑐ᩮ᦭⟤㧘 ㊁ፒᵗ᣿㧘 ⷏Ỉᱜ⼾㧘 ዊ㊁ኹℂ㧚 CADASIL㧘CARASILߩಽሶ∛ᘒᯏᐨ㧚 ᣣᧄ⥃ᐥ 2014;72:148-151.
12. ઁ↰ᱜ⟵㧘ᳰౝ ஜ㧚 HDLS㧚 කቇߩ޽ࠁߺ 2014;59:435
13. ዊ㊁ኹℂ㧚⣖ዊⴊ▤∛㧚 කቇߩ޽ࠁߺ 2014;59:436 
14. ㈬੗⋥ሶ㧘⍹ේᥓᒾ㧘ዊ㊁ኹℂ㧘⷏Ỉᱜ⼾. ALS10㧔TARDBPㆮવሶᄌ⇣ߦࠃࠆALS㧕. ೎ౠ ᣣᧄ⥃ῼ ᣂ㗔ၞ೎∝୥⟲ࠪ࡝࡯࠭, 2014;27:491-495
15. ᚻႦᢅਯ㧘 ⷏Ỉᱜ⼾㧘 ㊁ፒᵗ᣿㧘 ዊ㊁ኹℂ㧚 CADASIL㧘CARASILߩ∛ᘒᯏᐨ㧚 ⴊ▤කቇ
2014;15:51-58.
16. ㊁ ፒ ᵗ ᣿ 㧘 ⷏ Ỉ ᱜ ⼾ 㧘 ዊ ㊁ ኹ ℂ 㧚 CARASIL ߩ ᣂ ߒ ޿ ࠻ ࡇ ࠶ ࠢ ࠬ 㧚 ಽ ሶ ⣖ ⴊ ▤ ∛2014;13:179-181.
17. દ⮮ጪ‫⷏ޔ‬Ỉᱜ⼾‫ޔ‬ዊ㊁ኹℂ㧚Ἳ∝ߣ␹⚻ᄌᕈ㧚BRAIN MEDICAL

26(3): 259 -263 201418. ੹㊁ථ຦, ઁ↰ᱜ⟵, ઁ↰⌀ℂ, ⷏Ỉᱜ⼾, ᳰౝஜ. ゲ⚝ࠬࡈࠚࡠࠗ࠼ᒻᚑࠍ઻߁ㆮવᕈ߮߹ࠎᕈ
⊕⾰⣖∝ (HDLS) ߣ CSF-1R ㆮવሶᄌ⇣. BRAIN and NERVE-␹⚻⎇ⓥߩㅴᱠ, 2014;66:581590
19. ᮮ㑐᣿↵. ⌒⃿ㆇേᄬⴕߣૐࠕ࡞ࡉࡒࡦⴊ∝ࠍ઻߁ᣧ⊒ဳᄬ⺞∝ (EAOH) ߩ⥃ᐥ∛ဳߣಽሶ∛ᘒࡔࠞ࠾࠭ࡓߩ⸃᣿. ᣂẟකቇળ㔀⹹, 2014;128:1-6
20. ⍹ේᥓᒾ, ᩑ↰᣿⟤, 㜞ᯅဋ, ዊ㊁ኹℂ, ⷏Ỉᱜ⼾. ALS ߦ߅ߌࠆ spliceosome ⇣Ᏹ. ⥃ᐥ␹⚻ቇ, 2014;54:1155-1157
㩷㩷Φ ౒ห⎇ⓥ⎇ⓥ㗴⋡ ‫ޟ‬6)(Ǫࠪࠣ࠽࡞ߦᵈ⋡ߒߚ%#4#5+.ߩ↹ᦼ⊛ᴦ≮ᣇᴺߩᬌ⸛‫ޠ‬⎇ⓥౝኈ %#4#5+.ࡕ࠺࡞ࡑ࠙ࠬࠍ↪޿ߚಽሶᮡ⊛≮ᴺߩ㐿⊒ෳടᯏ㑐 ർ㉿ᄢቇ⎇ⓥ㗴⋡ ‫*ޟ‬VT#ᰳ៊ࡑ࠙ࠬߦ߅ߌࠆ⣖ዊⴊ▤ߩᯏ⢻⸃ᨆ‫ޠ‬⎇ⓥౝኈ %#4#5+.ࡕ࠺࡞ࡑ࠙ࠬߦ߅ߌࠆ⣖ⴊᵹߣⴊ▤∛ℂߩ⸃ᨆෳടᯏ㑐 ࿖┙ᓴⅣེ∛ᓴⅣེ∛⎇ⓥ࠮ࡦ࠲࡯

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 ᒰಽ㊁ߢߪ‫ޔ‬ᓥ᧪ߩᒻᘒቇ‫❱⚵∉఺ޔ‬ൻቇ‫↢ޔ‬ൻቇ⊛ಽᨆᴺߥߤߩᚻᴺࠍ↪޿ߚ␹⚻∛ℂቇߦട߃‫ޔ‬ฦ⒳⣖∔ᖚߩㆮવሶ࡟ࡌ࡞ߢߩ⸃ᨆࠍⴕߥߞߡ޿ࠆ‫⎇ޕ‬ⓥౝኈߣߒߡߪ‫ޔ‬⣖⣲≌ߩ∛࿃∛ᘒߩ
⎇ⓥࠍߪߓ߼‫∔ࠬ࡞ࠖ࠙ޔ‬ᖚ‫ޔ‬⣖⯯ⴊ‫⚻␹␹♖ޔ‬૞േ⮎ߥߤࠍኻ⽎ߣߒߚ⎇ⓥࠍⴕߥߞߡ޿ࠆ‫ޕ‬ ⣖⣲≌ߦ㑐ߒߡߪ‫ޔ‬ㄭᐕߢߪ‫≸ޔ‬ᛥ೙ㆮવሶࠍ%TGNQZ2ࠪࠬ࠹ࡓࠍ↪޿ߡ‫⚦ޔ‬⢩ㆬᛯ⊛ߦ⎕უߔࠆߎߣߦࠃࠆ⣖⣲≌ࡕ࠺࡞ࡑ࠙ࠬߩ∛ᘒⷰኤࠍ߅ߎߥߞߡ޿ࠆ߇‫ᧄޔ‬ᐕᐲߪ‫ࡁ࡞࠽࡚ࠪࠖ࠺ࡦࠦޔ‬࠶ࠢࠕ࠙࠻ᴺࠍ↪޿ߡ‫⚦ޔ‬⢩ㆬᛯ⊛ߥ2VEㆮવሶߩᯏ⢻⎕უ߇⣖⣲≌ߩ⊒↢ߦ❬߇ࠆߎߣታ㛎⊛ߦ⸽᣿ߒߚ‫ߚߒ↢⊒ޕ‬⣲≌ߪࡅ࠻㜑⧘⣲ߦ㘃ૃߒ‫ޔ‬ዊ⣖ේ⊒ߢ޽ߞߚ‫ޕ‬᠞⒳ߪ․ᓽ⊛ߢ‫ߩߘޔ‬᭽ᘒࠍ⸃ᨆਛߢ޽ࠆ‫࠻ࡅޔߚ߹ޕ‬⣖⣲≌ߩಽൻߦ߆߆ࠊࠆ⎇ⓥߢߪ‫৻ߩࡦࠞ࡝ࠣࠝ࠹ࡠࡊ㉄⎫ࡦ࡜ࡄࡋޔ‬⒳ߢ޽ࠆ)N[RKECP߇㗡⬄ౝ⢦⚦⢩ᕈ⣲≌‫ߦ․ޔ‬ෆ㤛ྙ⣲ߦ․ᓽ⊛ߦ⊒⃻ߔࠆߎߣࠍ᣿ࠄ߆ߦߒߚ‫ޕ‬ ߹ߚ‫⾰⊕Ⱞ)ޔ‬ᵴᕈဳౝะ߈ᢛᵹᕈࠞ࡝࠙ࡓ࠴ࡖࡀ࡞㧔)+4--KT㧕ߦኻߔࠆಽሶ⮎ℂቇ⊛⎇ⓥ߽⛮⛯ߒߡ޿ࠆ‫⚻␹ޔߪ࡞ࡀࡖ࠴ߩߎޕ‬વ㆐‛⾰‫౒⾰⊕Ⱞ)ޔ‬ᓎဳฃኈ૕ߣᯏ⢻⊛ߥ㑐ଥࠍᜬߜ‫ޔ‬␹⚻⚦⢩ߩ⥝ᅗᕈࠍ࠳ࠗ࠽ࡒ࠶ࠢߦ೙ᓮߔࠆ㊀ⷐߥࠗࠝࡦ࠴ࡖࡀ࡞ߢ޽ࠆ‫ᧄޕ‬ᐕᐲ߽‫ߩ∛ߟ߁ޔ‬ᴦ≮⮎ߣߒߡ↪޿ࠄࠇߡ޿ࠆ⮎೷ࠍਛᔃߦ‫)ޔ‬+4-࠴ࡖࡀ࡞߇ᛥ೙⊛ߦ௛޿ߡ޿ࠆߎߣࠍ‫࠷ࠞ࡝ࡈࠕޔ‬ࡔࠟࠛ࡞ෆᲣ⚦⢩Ⱞ⊕⾰⊒⃻♽ࠍ↪޿ߡ‫⸃ޔ‬ᨆߒߡ޿ࠆ‫ޕ‬Υ ⺰ᢥ㧔ේ⪺‫଀∝ޔ⺑✚ޔ‬ႎ๔ࠍ඙೎ߒߥ޿㧕 ᫪ ⑲᮸‫ޔ‬ᏒᎹን㓶‫ޔ‬ዊᨋ৻㓶‫ޔ‬㣐ጊ๺㓶㧦㗡⬄ౝේ⊒⢦⚦⢩ᕈ⣲≌ߦ߅ߌࠆ)N[RKECPߩ⊒⃻᭽ᑼ$TCKP6WOQT2CVJQNQI[ UWRRN R ᾢ⷏ᢅ㇢‫ޔ‬㋈ᧁ ⺰‫⧯ޔ‬ችንᶈ‫┻ޔ‬ਅጤ↵‫⭯ޔ‬੗ ብ‫ޔ‬㣐ጊ๺㓶‫ޔ‬ਣጊ ᥙ‫┻ޔ‬ౝᐘ⟤㧦ታ㛎⊛ࡑ࠙ࠬ⣖⣲≌⚦⢩ߩ⊒↢Უ⚦⢩㧔╙ႎ㧕㧦␹⚻ᐙ⚦⢩ࡑ࡯ࠞ࡯5QZ⊒⃻߆ࠄߺߚ⸃ᨆ
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