| Project/Area Number |
23591511
|
|---|
| Research Category |
Grant-in-Aid for Scientific Research (C)
|
| Allocation Type | Multi-year Fund |
|---|
| Section | 一般 |
|---|
| Research Field |
Pediatrics
|
|---|
| Research Institution | Osaka City University |
|---|
Principal Investigator |
TANAKA Akemi 大阪市立大学, 医学(系)研究科(研究院), 准教授 (30145776)
|
|---|
| Co-Investigator(Kenkyū-buntansha) |
NAEDA Mitsuyo 公益財団法人先端医療振興財団, 上級研究員 (40122080)
SETO Toshiyuki 大阪市立大学, 大学院医学研究科, 講師 (60423878)
SAWADA Tomo 大阪市立大学, 大学院医学研究科, 研究員 (60585991)
|
|---|
| Project Period (FY) |
2011 – 2013
|
| Project Status |
Completed (Fiscal Year 2013)
|
| Budget Amount *help |
¥5,200,000 (Direct Cost: ¥4,000,000、Indirect Cost: ¥1,200,000)
Fiscal Year 2013: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2012: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2011: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
|
|---|
| Keywords | ライソゾーム病 / 神経変性 / オートファジー / 経口治療薬 / 神経治療薬 |
|---|
| Research Abstract |
Autophagy is an important factor in the degeneration of neuronal cells of lysosomal storage diseases. We did an experimental study of modifying the autophagy to treat the neurodegeneration in the brain of the mucopolysaccharidosis type II model mouse (IDS-KO).
SCMAS staining of the brain of IDS-KO showed abnormal stimulation of autophagy in various cells especially in neuronal cells, which began from around 6 weeks old IDS-KO. At age 25 weeks, abnormal inclusion bodies appeared in the cytoplasm of neuronal cells of IDS-KO brain, which was not existed at age 20 weeks. We administered chloroquine to IDS-KO orally from 4 weeks old until the examination at 25 weeks old, which findings of the brain were similar to those of 20 weeks old IDS-KO. The result suggested that the inhibition of autophagy could suppress the disease progression in the brain of IDS-KO.
|
