Analysis of a novel hearing loss mechanism caused by mutated Cochlin
Project/Area Number |
15K14948
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Research Category |
Grant-in-Aid for Challenging Exploratory Research
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Allocation Type | Multi-year Fund |
Research Field |
Biological pharmacy
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Research Institution | The University of Tokyo |
Principal Investigator |
Yamamoto Kazoo 東京大学, 大学院新領域創成科学研究科, 教授 (20174782)
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Project Period (FY) |
2015-04-01 – 2017-03-31
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Project Status |
Completed (Fiscal Year 2016)
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Budget Amount *help |
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2016: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2015: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
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Keywords | 遺伝性疾患 / 難聴 / cochlin / グリコサミノグリカン / cochin |
Outline of Final Research Achievements |
Several missense mutations in the gene encoding Cochlin have been identified in pedigrees afflicted with late-onset non-syndromic autosomal-dominant hearing loss 9 (DFNA9). In this study, we demonstrated that Cochlin binds to sulfated glycosaminoglycan (GAG) and compared GAG-binding ability among wild-type and mutated Cochlins. Furthermore, we established anti-Cochlin monoclonal antibodies and analyzed localization of Cochlin and Cochlin ligands in inner ear by using monoclonal antibodies and Cochlin-Fc protein. Together with the data of quantitative analysis of Cochlin ligands accompanying aging and of Cochlin proteolysis by metalloproteinase showed a possible mechanism for understanding autosomal-dominant late-onset hearing loss in DFNA9 patients.
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Report
(3 results)
Research Products
(6 results)
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[Journal Article] Mutated leguminous lectin containing a heparin-binding like motif in a carbohydrate-binding loop specifically binds to heparin2015
Author(s)
Abo, H. , Soga, K., Tanaka, A., Tateno, H., Hirabayashi, J., Yamamoto, K
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Journal Title
PLoS One
Volume: 10
Issue: 12
Pages: 1-13
DOI
Related Report
Peer Reviewed / Open Access / Acknowledgement Compliant
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