Functional analyses of heparan sulfate degradation using Drosophila as a model for mucopolysaccharidosis
| Project/Area Number |
26840041
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| Research Category |
Grant-in-Aid for Young Scientists (B)
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| Allocation Type | Multi-year Fund |
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| Research Field |
Functional biochemistry
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| Research Institution | Tokyo Metropolitan Institute of Medical Science |
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Principal Investigator |
KAMIMURA Keisuke 公益財団法人東京都医学総合研究所, 脳発達・神経再生研究分野, 主席研究員 (30529524)
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| Project Period (FY) |
2014-04-01 – 2018-03-31
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| Project Status |
Completed (Fiscal Year 2017)
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| Budget Amount *help |
¥4,030,000 (Direct Cost: ¥3,100,000、Indirect Cost: ¥930,000)
Fiscal Year 2016: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2015: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2014: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
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| Keywords | ヘパラン硫酸 / プロテオグリカン / ムコ多糖代謝異常症 / ショウジョウバエ |
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| Outline of Final Research Achievements |
Heparan sulfate proteoglycans (HSPGs) are glycoproteins composed of a core protein with covalently attached heparan sulfate (HS) chains. HSPGs mediate interactions with a variety of extracellular ligands and participate in many physiological processes. In human, mutations in the lysosomal enzymes that degrade HS result in mucopolysaccharidosis, cause various organ dysfunction. Here, we examined the role of HS degradation during development using Drosophila melanogaster as a model system. We found that mutations in iduronidase, encoding a HS degradation enzyme, reduce the activity of BMP signaling and affect wing development.
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Report
(5 results)
Research Products
(17 results)
