Project/Area Number |
15K19610
|
Research Category |
Grant-in-Aid for Young Scientists (B)
|
Allocation Type | Multi-year Fund |
Research Field |
Pediatrics
|
Research Institution | Kyoto University |
Principal Investigator |
|
Project Period (FY) |
2015-04-01 – 2017-03-31
|
Project Status |
Completed (Fiscal Year 2016)
|
Budget Amount *help |
¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000)
Fiscal Year 2016: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Fiscal Year 2015: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
|
Keywords | iPS細胞 / 自己炎症性疾患 / サイトカイン / サイトカイン産生 / マウスモデル / iPS細胞由来単球 / 病態再現 |
Outline of Final Research Achievements |
We produced CD14-positive blood cells through iPS cells derived 3 MKD patients and 2 healthy controls. The MK activity of patient-derived blood cells was potently reduced and below 2% of healthy controls, so the phenotype of the peripheral blood cells was reproduced in iPS cell-derived blood cells. Next, we compared IL-1beta secretion from iPS-derived blood cells, but found no difference between parient-derived and control cells. We developed genetically-engineered mouse that express heterozygous mutant MVK gene. Then we bred them and obtained homozygous mutant mouse. We identified 10 Japanese MKD patients in total, including newly-diagnosed patients during the research period. We are preparing to submit a paper regarding the clinical and laboratory information.
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