Research Project
Grant-in-Aid for Challenging Exploratory Research
Severe congenital neutropenia (SCN) and cyclic neutropenia (CyN) are belonged to congenital bone marrow failure syndrome (CBMFS). Acute myelogenous leukemia (AML) was sometimes developed in the patients with some CBMFS including SCN. No AML occurred in the patients with CyN. To investigate the mechanism of AML occurrence, we established iPS cells from the patients (both had mutation in ELANE gene region) with SCN (SCN-iPS cells) or CyN (CyN-iPS cells), respectively. Myeloid cells derived from CyN-iPS cells revealed that the capability to differentiate into hematopoietic cells including neutrophils was almost similar to control iPS cells. SCN-iPS cells showed decreased capability into neutrophils, reflecting the disease status of SCN patients. To compare the mechanism of hematopoietic differentiation from SCN- and CyN-iPS cells was expected to be useful to analyze the mechanism of the onset of AML and treatment of these AML.
All 2015 2014
All Journal Article (6 results) (of which Peer Reviewed: 6 results, Open Access: 6 results) Presentation (1 results) Book (1 results)
Leukemia.
Volume: 29(3) Issue: 3 Pages: 606-14
10.1038/leu.2014.239
120007110642
Br J Haematol.
Volume: 164 Issue: 3 Pages: 451-464
10.1111/bjh.12638
Bone Marrow Transplant.
Volume: 49 Issue: 5 Pages: 634-9
10.1038/bmt.2014.10
Biol Blood Marrow Transplant.
Volume: 20 Issue: 4 Pages: 577-581
10.1016/j.bbmt.2013.12.563
PLos One
Volume: 9 Issue: 1 Pages: 1-10
10.1371/journal.pone.0087425
Leukemia
Volume: 28 Issue: 12 Pages: 2344-54
10.1038/leu.2014.136