| Item type |
デフォルトアイテムタイプ_（フル）(1) |
| 公開日 |
2006-03-21 |
| タイトル |
|
|
タイトル |
Defects of Granulopoiesis in Patients with Severe Congenital Neutropenia |
|
言語 |
en |
| 作成者 |
Nakamura, Kazuhiro
Kobayashi, Masao
Konishi, Nakao
Kawaguchi, Hiroshi
Miyagawa, Shin-ichiro
Sato, Takashi
Katoh, Osamu
Ueda, Kazuhiro
|
| 寄与者 |
|
|
|
姓名 |
国立情報学研究所 |
| アクセス権 |
|
|
アクセス権 |
open access |
|
アクセス権URI |
http://purl.org/coar/access_right/c_abf2 |
| 主題 |
|
|
主題Scheme |
Other |
|
主題 |
Granulocyte colony-stimulating factor |
| 主題 |
|
|
主題Scheme |
Other |
|
主題 |
Granulocyte colony-stimulating factor receptor |
| 主題 |
|
|
主題Scheme |
Other |
|
主題 |
Fluorescence activated cell sorting |
| 主題 |
|
|
主題Scheme |
Other |
|
主題 |
Severe congenital neutropenia |
| 主題 |
|
|
主題Scheme |
NDC |
|
主題 |
490 |
| 内容記述 |
|
|
内容記述 |
To confirm the abnormalities of primitive myeloid progenitor cells in patients withsevere congenital neutropenia (SCN), we studied their responsiveness to hematopoieticfactors including granulocyte colony-stimulating factor (G-CSF). In all SCN patientsstudied no abnormalities of granulocyte colony-stimulating factor receptor (G-CSFR) genewere detected by polymerase chain reaction-single-strand conformation polymorphism analysisand sequence analysis. A flow cytometric analysis of bone marrow cells based on theexpression of CD34, Kit receptor, and G-CSFR demonstrated a reduced frequency ofCD34+/Kit+/G-CSFR+ cells in patients with SCN. The granulocyte/macrophage (GM)-colonyformation of CD34+/Kit+/G-CSFR+ cells in patients was markedly decreased at allconcentrations of G-CSF in serum-deprived semisolid culture. The responsiveness ofCD34+/Kit+/G-CSFR+ cells in patients showed a reduced response to the combination of stemcell factor, the ligand for flk2/flt3, and interleukin-3 with or without G-CSF inserum-deprived semisolid and liquid suspension cultures. In contrast, no difference in theresponsiveness of CD34+/Kit+/G-CSFR- cells was noted between SCN patients and normalsubjects. The bone marrow cells from a patient who underwent bone marrow transplantationshowed a restoration of both the reduced frequency and the decreased level of GM-colonyformation of CD34+/Kit+/G-CSFR+ cells. These results demonstrate that the presence ofqualitative and quantitative abnormalities of primitive myeloid progenitor cells expressingG-CSFR may play an important role in the impairment of granulopoiesis in patients with SCN. |
|
言語 |
en |
| 出版者 |
|
|
出版者 |
Hiroshima University Medical Press |
| 日付 |
|
|
日付 |
2006-03-21 |
|
日付タイプ |
Created |
| 言語 |
|
|
言語 |
eng |
| 資源タイプ |
|
|
資源タイプ識別子 |
http://purl.org/coar/resource_type/c_6501 |
|
資源タイプ |
departmental bulletin paper |
| 出版タイプ |
|
|
出版タイプ |
VoR |
|
出版タイプResource |
http://purl.org/coar/version/c_970fb48d4fbd8a85 |
| 収録物識別子 |
|
|
収録物識別子タイプ |
ISSN |
|
収録物識別子 |
0018-2052 |
| 収録物識別子 |
|
|
収録物識別子タイプ |
NCID |
|
収録物識別子 |
AA00664312 |
| 開始ページ |
|
|
開始ページ |
63 |
| 書誌情報 |
Hiroshima Journal of Medical Sciences
Hiroshima Journal of Medical Sciences
巻 51,
号 3,
p. 63-74,
発行日 2002-09-10
|
| 旧ID |
280 |
KJ00000010488.pdf (1.5 MB)
