| 研究実績の概要 |
D4ST-1-deficient Ehlers-Danlos syndrome (DD-EDS, Kosho Type) is a rare heterogeneous connective tissue disorder. Patients suffer from serious subcutaneous hematoma which is possible caused by insufficient contraction of the arterial smooth muscle. In this project, we investigate mechanism of hematoma using with patient-specific iPS cell-derived vessels smooth muscle cells (VSMCs).
In research plan for 2014, I made a plane to identify functional iPS cell-derived VSMCs, observe contractile protein, and contraction function. I assay the gene expression of contractile protein.
Besides, the conviction for disease mechanism from the only one patient specific iPS cells is not so strong, so we made one more DD-EDS specific iPS cells from the other patient.
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| 現在までの達成度 (区分) |
現在までの達成度 (区分)
2: おおむね順調に進展している
理由
According to the application from innitially planned, I should identify functional iPS cell-derived VSMCs, observe contractile protein, contractile in response to pharmacological agonists such as carbachol or KCl, and vessel formation in matrigel plugs.
During 2014, I induced the VSMCs from patient specific-iPS cells, and compared the relative gene expression of VSMC contraction, contractile protein expression in teratoma formed from patient iPS cells. Besides, we established DD-EDS iPS cells from the other patient, so that the final results become more convictive. Moreover, we induced the VSMCs from this DD-EDS iPS cells. Therefore, the contraction function assay was delayed.
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| 今後の研究の推進方策 |
Since I have induced the differentiation of VSMCs from two patient-specific iPS cells successfully, in the following 2015 year, I plan to do contraction function assay, and then make vessel in matrigel plugs. If possible, I will also do drug screening in vitro, such as growth factors.
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