The pathological analysis of neutropenia using by iPS cells

• Project/Area Number: 26870396
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Immunology; Pediatrics
• Research Institution: Hiroshima University
• Principal Investigator: Karakawa Shuhei 広島大学, 医歯薬保健学研究院, 助教 (10642150)
• Project Period (FY): 2014-04-01 – 2016-03-31
• Project Status: Completed (Fiscal Year 2015)
• Budget Amount: ¥4,030,000 (Direct Cost: ¥3,100,000、Indirect Cost: ¥930,000); Fiscal Year 2015: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000); Fiscal Year 2014: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
• Keywords: 好中球減少症 / ELANE / 重症先天性好中球減少症 / 周期性好中球減少症 / エラスターゼ / Biｐ / アポトーシス / iPS細胞

Outline of Final Research Achievements

We established iPS cells derived from health donor (WT-iPS), severe congenital neutropenia (SCN), and cyclic neutropenia (CyN). And we established differentiation therapy from iPS cells to hematopoietic stem cells and granuloccytes without serum and feeder cells.
The colony formation and cell proliferation of CD34 positive cells derived from SCN-iPS were decreased compared with WT-iPS or CyN-iPS, suggesting the possibility that the ability of hematopoiesis and proliferation were impaired in the early stage of hemoangiogenic progenitor. There were no definite difference in the cell localization of elastase and the expression of BIP protein between SCN-iPS, CyN-iPS and WT-iPS. These date suggest that the impairment in the early proliferation stage cause the severe neutropenia.