1997 Fiscal Year Final Research Report Summary
Analysis of gene defects related to NK cell deficiency : Establishment of the disorder as a new immunodeficiency
| Project/Area Number |
08457222
|
|---|
| Research Category |
Grant-in-Aid for Scientific Research (B)
|
| Allocation Type | Single-year Grants |
|---|
| Section | 一般 |
|---|
| Research Field |
Pediatrics
|
|---|
| Research Institution | Shinshu University |
|---|
Principal Investigator |
KOMIYAMA Atsushi Shinshu Univ.School Med., Dept.Pediatr., Professor, 医学部, 教授 (50020798)
|
|---|
| Co-Investigator(Kenkyū-buntansha) |
AGEMATSU Kazunaga Shinshu Univ.Hosp., Dept.Pediatr., Associate Professor, 医学部附属病院, 講師 (60262721)
KOIKE Kenichi Shinshu Univ.School Med., Dept.Pediatr., Assistant Professor, 医学部, 助教授 (40143979)
|
|---|
| Project Period (FY) |
1996 – 1997
|
| Keywords | NK cell / NK cell deficiency / familial NK cell deficiency / Chediak-Higashi syndrome / perforin / Fas / Fas ligand / immunodericiency |
|---|
| Research Abstract |
The present studies were conducted to analyze the clinical chatacteristics and gene defects of natural killer (NK) cell deficiencies.
1. Familial NK cell deficieny (Komiyama, A., Pediatr., 85 : 323,1990)
(1) The hypersusceptibility to infections appears to be improved with age, with a gradual increase in the number of circulating rdT cells.
(2) The patients have CD56+ cells, all of which are CD3+CD56+ cells but not CD3-CD56+ cells. These results indicate that this disorder is due to the absence of circulating NK cells.
(3) Then, next studies were performed to examine the proliferative capacity of NK cell precursors by culturing the bone marrow cells in vitro. We failed to culture CD3-CD56+ cells in the presence of a combination of many cytokines or feeder layrs + IL-2 in the culture system. Further studies are necessary to elucidate the defects.
(4) The expression of perforin mRNA was normal in the cytotoxic T cells.
2. Chediak-Higashi syndrome
(1) NK cell activity was defective against K562 cells but almost normal against Jurkat cells. The latter activity was not exerted by tumor necrosis factor-a.
(2) The perfor in-mediated cytotoxicity was impaired, but its mRNA was normally expressedin the NK cells.
(3) The Fas mRNA expression was normal, and Fas/Fas ligand-mediated cytotoxicity via apoptosis was normal.
3.Other disorders such as hemophagocytic syndrome, systemic lupus erythematosus, and NK cell abnormality related to the Chernobyl accident.
The NK cell deficiencies in these disordeers were similarly analyzed in the present studies.
|
