2000 Fiscal Year Final Research Report Summary
Study of lysosomal storage disease-intracellular signaling and apoptosis
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 |
Section | 一般 |
Research Field |
Pediatrics
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Research Institution | Osaka University |
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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Keywords | Farber disease / Subcutaneous nodules / apoptosis / Ceramide / MCP-1 / TGF-β |
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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Research Products
(15 results)
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[Publications] Akagi M, Inui K, Nishigaki T, Muramatsu T, Kokubu C, Fu L, Fukushima H, Yanagihara I, Tsukamoto H, Kurahashi H, Okada S: "Mutation analysis of a Japanese patient with fucosidosis."J Hum Genet. 44. 323-326 (1999)
Description
「研究成果報告書概要(欧文)」より
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[Publications] Yamamoto T, Nanba E, Ninomiya H, Higaki K, Taniguchi M, Zhang H, Akaboshi S, Watanabe Y, Takeshima T, Inui K, Okada S, Tanaka A, Sakuragawa N, Millat G, Vanier MT, Morris JA, Pentchev PG, Ohno K: "NPC1 gene mutations in Japanese patients with Niemann-Pick disease type C."Hum Genet. 105. 10-16 (1999)
Description
「研究成果報告書概要(欧文)」より
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