2023 Fiscal Year Final Research Report
The phenotype of citrin-KO mice by cystine reduced nutrition
| Project/Area Number |
20K16844
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| Research Category |
Grant-in-Aid for Early-Career Scientists
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| Allocation Type | Multi-year Fund |
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| Review Section |
Basic Section 52050:Embryonic medicine and pediatrics-related
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| Research Institution | Tohoku University (2023)
Nihon University (2020-2022) |
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Principal Investigator |
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| Project Period (FY) |
2020-04-01 – 2024-03-31
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| Keywords | システイン / シスチン / シトルリン / ミトコンドリア / ATP |
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| Outline of Final Research Achievements |
This study aimed to explore the pathogenesis of neonatal intrahepatic cholestasis caused by citrin deficiency, NICCD, related to cysteine. The phenotype of citrin KO mice fed different cysteine concentrations was assessed. There were no changes in survival, appearance, body weight, or blood level of AST/ALT between each group of mice. Analysis of liver pathological characteristics is proceeding. We need further studies to elucidate the pathogenesis of NICCD.
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| Free Research Field |
先天代謝異常
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| Academic Significance and Societal Importance of the Research Achievements |
シトリン欠損症はアジアで比較的頻度の高い疾患で、その詳しい病態については未解明な点も多い。今回、システイン代謝と本疾患との関連に着目し解析を進めたが、システイン含有濃度の異なる飼料を与えたシトリンKOマウスにおいては明らかな表現型の変化は認めなかった。種間におけるシステイン代謝の差異なども考慮されるため、今後、解析対象細胞を変えるなど、さらなる研究が望まれる。
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