2011 Fiscal Year Final Research Report
Toxicity of sugars and identification of markers for pathophysiology in citrin deficiency leading to development of novel therapeutics
| Project/Area Number |
21591337
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Pediatrics
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| Research Institution | Kumamoto University (2011)
Tokushima Bunri University (2009-2010) |
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Principal Investigator |
SAHEKI Takeyori 熊本大学, 生命資源研究・支援センター, 特任教授 (10056070)
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| Co-Investigator(Kenkyū-buntansha) |
KOBAYASHI Keiko 鹿児島大学, 大学院・医歯学総合研究科, 准教授 (70108869)
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| Project Period (FY) |
2009 – 2011
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| Keywords | シトリン / 成人発症II型シトルリン血症(CTLN2) / 新生児肝内胆汁うっ滞症(NICCD) / マウスモデル / 糖質毒性 / メタボローム解析 |
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| Research Abstract |
We have established a model mouse of citrin deficiency causing neonatal intrahepatic cholestasis(NICCD) at neonatal period and adult-onset type II citrullinemia(CTLN2) at adult age. We showed that administration of sugars to the mice caused deterioration such as hyperammonemia. From the metabolomics analysis, we found that administration of sugars induced a marked increase in hepatic glycerol-3-phosphate(G3P), which is useful as a biomarker for citrin deficiency. We found that dietary change from high protein to low protein induced a decreased body weight, mimicking growth retardation or skinniness in the disease and that addition of substance such as protein, amino acids, pyruvate and medium-chain triglyceride to the low-protein diet increased food intake and body weight.
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[Journal Article] Genotypic and phenotypic features of citrin deficiency : five-year experience in a Chinese pediatric center2011
Author(s)
Song YZ, Deng M, Chen FP, Wen F, Guo L, Cao SL, Gong J, Xu H, Jiang GY, Zhong L, Kobayashi K, Saheki T, Wang ZN
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Journal Title
Int J Mol Med
Volume: 28(1)
Pages: 33-40
DOI
Peer Reviewed
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