2013 Fiscal Year Final Research Report
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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| 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 |
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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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Research Products
(7 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
Pages: 986462
DOI
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 Diabetes2012
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
Pages: e49055
DOI
Peer Reviewed
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[Journal Article] Simultaneous two-dimensional HPLC determination of free d-serine and d-alanine in the brain and periphery of mutant rats lacking d-amino-acid oxidase2011
Author(s)
Miyoshi, Y., Hamase, K., Okamura, T., Konno, R., Kasai, N., Tojo, Y. and Zaitsu, K
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Journal Title
J Chromatogr B Analyt Technol Biomed Life Sci
Volume: 879
Pages: 3184-3189
DOI
Peer Reviewed
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