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  1. 070 農学部, 大学院農学研究科
  2. 0701 学術論文

Transgenic Monkey Model of the Polyglutamine Diseases Recapitulating Progressive Neurological Symptoms

http://hdl.handle.net/10091/00021212
http://hdl.handle.net/10091/00021212
12edf33b-833e-408c-a81a-08ed0b5a3808
名前 / ファイル ライセンス アクション
26870884_01.pdf 26870884_01.pdf (3.6 MB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2019-02-15
タイトル
言語 en
タイトル Transgenic Monkey Model of the Polyglutamine Diseases Recapitulating Progressive Neurological Symptoms
言語
言語 eng
キーワード
主題Scheme Other
主題 Neurodegenerative disease
キーワード
主題Scheme Other
主題 Polyglutamine disease
キーワード
主題Scheme Other
主題 Transgenic monkey
キーワード
主題Scheme Other
主題 Common marmoset
資源タイプ
資源 http://purl.org/coar/resource_type/c_6501
タイプ journal article
著者 Tomioka, Ikuo

× Tomioka, Ikuo

WEKO 107370

Tomioka, Ikuo

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Ishibashi, Hidetoshi

× Ishibashi, Hidetoshi

WEKO 107371

Ishibashi, Hidetoshi

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Minakawa, Eiko N.

× Minakawa, Eiko N.

WEKO 107372

Minakawa, Eiko N.

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Motohashi, Hideyuki H.

× Motohashi, Hideyuki H.

WEKO 107373

Motohashi, Hideyuki H.

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Takayama, Osamu

× Takayama, Osamu

WEKO 107374

Takayama, Osamu

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Saito, Yuko

× Saito, Yuko

WEKO 107375

Saito, Yuko

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Popiel, H. Akiko

× Popiel, H. Akiko

WEKO 107376

Popiel, H. Akiko

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Puentes, Sandra

× Puentes, Sandra

WEKO 107377

Puentes, Sandra

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Owari, Kensuke

× Owari, Kensuke

WEKO 107378

Owari, Kensuke

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Nakatani, Terumi

× Nakatani, Terumi

WEKO 107379

Nakatani, Terumi

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Nogami, Naotake

× Nogami, Naotake

WEKO 107380

Nogami, Naotake

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Yamamoto, Kazuhiro

× Yamamoto, Kazuhiro

WEKO 107381

Yamamoto, Kazuhiro

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Noguchi, Satoru

× Noguchi, Satoru

WEKO 107382

Noguchi, Satoru

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Yonekawa, Takahiro

× Yonekawa, Takahiro

WEKO 107383

Yonekawa, Takahiro

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Tanaka, Yoko

× Tanaka, Yoko

WEKO 107384

Tanaka, Yoko

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Fujita, Naoko

× Fujita, Naoko

WEKO 107385

Fujita, Naoko

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Suzuki, Hikaru

× Suzuki, Hikaru

WEKO 107386

Suzuki, Hikaru

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Kikuchi, Hisae

× Kikuchi, Hisae

WEKO 107387

Kikuchi, Hisae

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Aizawa, Shu

× Aizawa, Shu

WEKO 107388

Aizawa, Shu

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Nagano, Seiichi

× Nagano, Seiichi

WEKO 107389

Nagano, Seiichi

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Yamada, Daisuke

× Yamada, Daisuke

WEKO 107390

Yamada, Daisuke

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Nishino, Ichizo

× Nishino, Ichizo

WEKO 107391

Nishino, Ichizo

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Ichinohe, Noritaka

× Ichinohe, Noritaka

WEKO 107392

Ichinohe, Noritaka

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Wada, Keiji

× Wada, Keiji

WEKO 107393

Wada, Keiji

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Kohsaka, Shinichi

× Kohsaka, Shinichi

WEKO 107394

Kohsaka, Shinichi

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Nagai, Yoshitaka

× Nagai, Yoshitaka

WEKO 107395

Nagai, Yoshitaka

Search repository
Seki, Kazuhiko

× Seki, Kazuhiko

WEKO 107396

Seki, Kazuhiko

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信州大学研究者総覧へのリンク
氏名 Tomioka, Ikuo
URL http://soar-rd.shinshu-u.ac.jp/profile/ja.WNAhPUkh.html
出版者
出版者 Society for Neuroscience
引用
内容記述タイプ Other
内容記述 eNeuro.4(2):e0250(2017)
書誌情報 eNeuro

巻 4, 号 2, p. e0250, 発行日 2017-03-28
抄録
内容記述タイプ Abstract
内容記述 Age-associated neurodegenerative diseases, such as Alzheimer’s disease, Parkinson’s disease, and the polyglutamine (polyQ) diseases, are becoming prevalent as a consequence of elongation of the human lifespan. Although various rodent models have been developed to study and overcome these diseases, they have limitations in their translational research utility owing to differences from humans in brain structure and function and in drug metabolism. Here, we generated a transgenic marmoset model of the polyQ diseases, showing progressive neurological symptoms including motor impairment. Seven transgenic marmosets were produced by lentiviral introduction of the human ataxin 3 gene with 120 CAG repeats encoding an expanded polyQ stretch. Although all offspring showed no neurological symptoms at birth, three marmosets with higher transgene expression developed neurological symptoms of varying degrees at 3–4 months after birth, followed by gradual decreases in body weight gain, spontaneous activity, and grip strength, indicating time-dependent disease progression. Pathological examinations revealed neurodegeneration and intranuclear polyQ protein inclusions accompanied by gliosis, which recapitulate the neuropathological features of polyQ disease patients. Consistent with neuronal loss in the cerebellum, brain MRI analyses in one living symptomatic marmoset detected enlargement of the fourth ventricle, which suggests cerebellar atrophy. Notably, successful germline transgene transmission was confirmed in the second-generation offspring derived from the symptomatic transgenic marmoset gamete. Because the accumulation of abnormal proteins is a shared pathomechanism among various neurodegenerative diseases, we suggest that this new marmoset model will contribute toward elucidating the pathomechanisms of and developing clinically applicable therapies for neurodegenerative diseases.
資源タイプ(コンテンツの種類)
内容記述タイプ Other
内容記述 Article
ISSN
収録物識別子タイプ ISSN
収録物識別子 2373-2822
PubMed
識別子タイプ PMID
関連識別子 https://pubmed.ncbi.nlm.nih.gov/28374014/
関連名称 28374014
DOI
識別子タイプ DOI
関連識別子 https://doi.org/10.1523/ENEURO.0250-16.2017
関連名称 10.1523/ENEURO.0250-16.2017
権利
権利情報 Copyright © 2017 Tomioka et al. This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 International license, which permits unrestricted use, distribution and reproduction in any medium provided that the original work is properly attributed.
出版タイプ
出版タイプ VoR
出版タイプResource http://purl.org/coar/version/c_970fb48d4fbd8a85
WoS
表示名 Web of Science
URL http://gateway.isiknowledge.com/gateway/Gateway.cgi?&GWVersion=2&SrcAuth=ShinshuUniv&SrcApp=ShinshuUniv&DestLinkType=FullRecord&DestApp=WOS&KeyUT=000397567900013
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