2002 Fiscal Year Final Research Report Summary
GENE EXPRESSIONS DURING PROLIFERATION AND DIFFERENTIATION OF MYELOID CELIS IN SEVERE CONCENITAL NEUTROPENIA
| Project/Area Number |
13670806
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Pediatrics
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| Research Institution | HIROSHIMA UNICERSITY |
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Principal Investigator |
KOBAYASHI Masao Hiroshima University Graduate School of Education, Professor, 大学院・教育学研究科, 教授 (00162016)
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| Co-Investigator(Kenkyū-buntansha) |
KOTOH Osamu Hiroshima University Research Institute for Radiation Biology, Associate Professor, 原爆放射線医科学研究所, 助教授 (90214361)
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| Project Period (FY) |
2001 – 2002
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| Keywords | Sever congenital neutropenia / Myeloid proliferation / Gene expression / Neutrophil elastase / Primary granules |
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| Research Abstract |
Recently, mutations in the neutrophil elastase (NE) gene, ELA2, have been reported in patients with severe congenital Neutrogena (SCN). We studied the expression of granule constituent genes in primitive myeloid progenitor cells during proliferation and differentiation in patients with SCN. Primitive myeloid cells in the bone marrow were purified based on the expression of CD34 and G-CSF receptor (G-CSFR). The CD34^+ / G-CSFR^+ cells, but not the CD34^+ / G-CSFR cells, of SCN patients showed a reduced number of GM-colony formation and defectives proliferation in response to G-CSF alone and to the combinations of hematopoietic factors in serumdeprived culture. The CD34^+ / G-CSFR^+ cells expressed low levels of the granule constituent mRNAs. The transcription levels of primary granule enzyme genes were gradually enhanced and then decreased when cells were induced toward myeloid lineage in the presence of G-CSF in normal subjects. However, the up-regulation of the transcription in the early stage of proliferation of SCN patients was significantly lower than those of normal subjects. Furthermore, the down-regulation of these enzyme transcriptions was not clearly observed in SCN patients. No differences in the expressions of lactoferrin gene were seen between normal subjects and patients with SCN. These results suggest that the abnormal regulation of the transcription in primary granule consitituents may play a potential role in the defective proliferation and differentiation of myeloid lineage in patients with SCN.
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Research Products
(12 results)
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[Publications] Kawaguchi H., Kobayashi M., Nakamura N., Konishi N., Miyagawa S., Sato T., Toyota H., Komada Y., Kojima S., Todoroki Y., Ueda K. & Katoh O: "Dysregulation of transcriptions in primary granule constituents during myeloid proliferation and differentiation in patients with severe congenital neutropenia"Journal of Leukocyte Biology. 73. 225-234 (2003)
Description
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