Therapeutic strategy for Niemann-Pick disease type C focusing on induction of ACAT1-positive late endosomes
| Project/Area Number |
23590448
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Experimental pathology
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| Research Institution | The University of Tokushima |
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Principal Investigator |
SAKASHITA Naomi 徳島大学, ヘルスバイオサイエンス研究部, 教授 (90284752)
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| Co-Investigator(Kenkyū-buntansha) |
TAKEYA Motohiro 熊本大学, 大学院生命科学研究部, 教授 (90155052)
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| Project Period (FY) |
2011-04-28 – 2015-03-31
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| Project Status |
Completed (Fiscal Year 2014)
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| Budget Amount *help |
¥5,200,000 (Direct Cost: ¥4,000,000、Indirect Cost: ¥1,200,000)
Fiscal Year 2013: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2012: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2011: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
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| Keywords | C型Niemann-Pick病 / マクロファージ / ACAT1 / 後期エンドゾーム / コレステロール / NPC1 / コレステロールエステル |
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| Outline of Final Research Achievements |
Niemann-Pick disease type C (NPC1-/-) is lysosomal storage disease resulting from deficiency of intracellular cholesterol trafficking protein NPC1, which accumulates massive cholesterol in late endosome and cause cellular/tissue injury. We tried to rescue NPC1-/- employing induction of ACAT1-positive late endosomes (ACAT1-LE) to the NPC1-/- mice. Induction of ACAT1-LE to NPC1-/- macrophages resulted in decrease of free cholesterol and increase of cholesteryl ester. Induction of ACAT1-LE to NPC1-/- neonates by administration of methyl-beta-cycrodextrin-cholesterol complex improved life span of the mice. These results suggested that efficient ACAT1-LE induction is essential for NPC-/- therapeutic strategy.
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Report
(5 results)
Research Products
(70 results)
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[Journal Article] Concomitant accumulation of α-synuclein and TDP-43 in a patient with corticobasal degeneration.2014
Author(s)
Yamashita S, Sakashita N, Yamashita T, Tawara N, Tasaki M, Kawakami K, Komohara Y, Fujiwara Y, Kamikawa M, Nakagawa T, Hirano T, Maeda Y, Hasegawa M, Takeya M, Ando Y
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Journal Title
Journal of Neurology
Volume: 261
Issue: 11
Pages: 2209-2217
DOI
Related Report
Peer Reviewed / Open Access
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[Journal Article] Constrictive pericarditis with intrapericardial abscess.2013
Author(s)
Hara T, Yamada H, Takashima A, Yamasaki H, Ogasawara K, Bando S, Ise T, Niki T, Kusunose K, Ueda Y, Tomita N, Yamaguchi K, Taketani Y, Iwase T, Soeki T, Wakatsuki T, Horiguchi H, Sakashita N, Sata M
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Journal Title
Circulation Jorunal
Volume: 77(3)
Pages: 786-788
NAID
Related Report
Peer Reviewed
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[Journal Article] Marked cardiomegaly in a patient with familial amyloidotic polyneuropathy after orthotopic liver transplantation: a case study2013
Author(s)
Sakashita N, Kagawa S, Date R, Ueno T, Nakagawa T, Yamashita T, Obayashi K, Ohshima T, Ueda M, Horiguchi H, Ando Y, Takeya M
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Journal Title
Pathol Int
Volume: 63
Issue: 5
Pages: 260-265
DOI
Related Report
Peer Reviewed
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[Presentation] 頸部皮下腫瘍2014
Author(s)
中須千春、堀口英久、坂下直実
Organizer
第114回中国四国スライドカンファレンス
Place of Presentation
高知大学、高知県、南国市
Year and Date
2014-06-14
Related Report
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[Presentation] 皮膚腫瘍。2013
Author(s)
大本卓司、吉谷信幸、堀口英久、坂下直実
Organizer
第111回中国四国スライドカンファレンス
Place of Presentation
松江テルサ、島根県、松江市
Related Report
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[Presentation] 膵腫瘍。2013
Author(s)
吉谷信幸、大本卓司、堀口英久、坂下直実
Organizer
第112回中国四国スライドカンファレンス
Place of Presentation
岡山大学Jホール、岡山県、岡山市
Related Report
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[Presentation] 子宮腫瘍2012
Author(s)
坂下直実 他
Organizer
第108回中国･四国スライドカンファレンス
Place of Presentation
川崎医科大学、岡山県
Related Report
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[Presentation] 大腸腫瘍2012
Author(s)
香川聖子 他
Organizer
第109回中国四国スライドカンファレンス
Place of Presentation
広島西医療センター、広島県
Related Report
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