| Project/Area Number |
11670760
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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 | Osaka University |
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Principal Investigator |
INUI Koji Osaka University Graduate School of Medicine, Associate Professor, 医学系研究科, 助教授 (90175208)
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| Co-Investigator(Kenkyū-buntansha) |
酒井 規夫 大阪大学, 医学系研究科, 助手 (30314313)
OKADA Shintaro Osaka University Graduate School of Medicine, Professor, 医学系研究科, 教授 (30028609)
TSUKAMOTO Hiroko Osaka University Graduate School of Medicine, Assostant Professor, 医学系研究科, 助手 (50263281)
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| Project Period (FY) |
1999 – 2000
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| Project Status |
Completed (Fiscal Year 2000)
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| Budget Amount *help |
¥3,600,000 (Direct Cost: ¥3,600,000)
Fiscal Year 2000: ¥1,400,000 (Direct Cost: ¥1,400,000)
Fiscal Year 1999: ¥2,200,000 (Direct Cost: ¥2,200,000)
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| Keywords | Farber disease / Subcutaneous nodules / apoptosis / Ceramide / MCP-1 / TGF-β / 炎症性サイトカイン / RT-PCR法 / リソソーム病 / 脂質蓄積 |
|---|
| Research Abstract |
Farber disease is a rare inherited metaboric disorder, in which cardinal symptom is ceramide enriched subcutaneous nodules due to a deficiency of lysosomal acid ceramidase. I examined expressed genes in nodules from a patient and macrophage like cell lines induced by cell permeable ceramide, to elucidate the roll of ceramide in cell signaling mechanism. From these results, I deduced that ceramide accumulation induced macrophage infiltration in nodules through the up-regulation of the MCP-1 (macrophage chemoattractant protein 1) gene expression and also induced TGF-β gene expression. These results partly explain the pathogenesis of nodule formation and macrophage infiltration.
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