Establishment of a novel rat model for cystinosis
| Project/Area Number |
23500508
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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 |
Laboratory animal science
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| Research Institution | Research Institute, International Medical Center of Japan |
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Principal Investigator |
OKAMURA TADASHI 独立行政法人国立国際医療研究センター, その他部局等, その他 (00333790)
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| Co-Investigator(Renkei-kenkyūsha) |
SHIMIZU Yukiko (独)国立国際医療研究センター研究所, 動物実験施設, 技術職員 (00469983)
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| Project Period (FY) |
2011 – 2013
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| Project Status |
Completed (Fiscal Year 2013)
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| Budget Amount *help |
¥5,200,000 (Direct Cost: ¥4,000,000、Indirect Cost: ¥1,200,000)
Fiscal Year 2013: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2012: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2011: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
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| Keywords | 疾患モデル / シスチン蓄積症 / シスチノーシス / リソソーム蓄積症 / HPLC / モデルラット / (2) モデルラット / (3) HPLC |
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| Research Abstract |
Cystinosis is a rare lysosomal storage disease characterized by the abnormal accumulation of cysteine in various organs. Accumulation of cysteine in all tissues eventually leads to multisystemic disease. The causative gene, CTNS, encodes cystinosin, the lysosomal cysteine transporter. We identified a 13-bp deletion within Ctns gene in LEA rat, and have established a congenic strain carrying mutant Ctns gene from LEA rat on F344 genetic background. We also developed a simple and rapid method for determination of cysteine using HPLC to evaluate its accumulation in cells and tissues. The congenic rats lacking the function of Ctns is expected to be a useful for model animal of understanding cystinosis.
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Report
(4 results)
Research Products
(11 results)
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[Journal Article] Phenotypic Characterization of LEA Rat: A New Rat Model of Nonobese Type 2 Diabetes2013
Author(s)
Okamura, T., Pei, XY., Miyoshi, I., Shimizu, Y., Takanashi-Yanobu, R., Mototani, Y., Takao Kanai, T., Satoh, J., Kimura, N. and Kasai, N
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Journal Title
J Diabetes Res
Volume: 2013
Pages: 986462-986462
DOI
Related Report
Peer Reviewed
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[Journal Article] Serotonin regulates glucose-stimulated insulin secretion from pancreatic ß cells during pregnancy.2013
Author(s)
Ohara-Imaizumi, M., Kim, H., Yoshida, M., Fujiwara, T., Aoyagi, K., Toyofuku, Y., Nakamichi, Y., Nishiwaki, C., Okamura, T., Uchida, T., Fujitani, Y., Akagawa, K., Kakei, M., Watada, H., German, M.S., and Nagamatsu, S.
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Journal Title
Proc. Natl. Acad. Sci. USA
Volume: 110
Issue: 48
Pages: 19420-19425
DOI
Related Report
Peer Reviewed
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[Journal Article] Quantitative assessment of pdx1 promoter activity in vivo using a secreted luciferase reporter system.2013
Author(s)
Nishimura, W., Eto, K., Miki, A., Goto, M., Kawaguchi, M., Nammo, T., Udagawa, H., Hiramoto, M., Shimizu, Y., Okamura, T., Fujiwara, T., Yasuda, Y. and Yasuda, K.
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Journal Title
Endocrinology
Volume: 154
Issue: 11
Pages: 4388-4395
DOI
Related Report
Peer Reviewed
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[Journal Article] Deletion of CDKAL1 Affects High-Fat Diet-Induced Fat Accumulation and Glucose-Stimulated Insulin Secretion in Mice, Indicating Relevance to Diabetes.2012
Author(s)
Okamura T, Yanobu-Takanashi R, Takeuchi F, Isono M, Akiyama K, Shimizu Y, Goto M, Liang Y-Q, Yamamoto K, Katsuya T, Fujioka A, Ohnaka K, Takayanagi R, Ogihara T, Yamori Y, Kato N.
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Journal Title
PLoS One
Volume: 7
Issue: 11
Pages: e49055-e49055
DOI
Related Report
Peer Reviewed
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