| 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)
|
|---|
| 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.
|
