2013 Fiscal Year Final Research Report
Studies on regulation of structure and function of primary cilia by tubulin polyglutamylation
| Project/Area Number |
23570209
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Molecular biology
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| Research Institution | Hamamatsu University School of Medicine |
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Principal Investigator |
IKEGAMI KOJI 浜松医科大学, 医学部, 准教授 (20399687)
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| Research Collaborator |
KONNO Alu 日本学術振興会, 特別研究員PD
HANG Vu Thi 浜松医科大学, 技術補佐員
OGAWA Miya 浜松医科大学, 技術補佐員
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| Project Period (FY) |
2011 – 2013
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| Keywords | 翻訳後修飾 / 繊毛病 / 微小管 / チューブリン / 繊毛 / 軸糸 |
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| Research Abstract |
In this study, we aimed to identify enzymes that regulated tubulin polyglutamylation in primary cilia. We have finally identified three enzymes, TTLL1, TTLL6, and CCP1, as polyglutamylation-regulating enzymes in primary cilia. We found, by blocking the localization of TTLL6 into primary cilia or by the direct inhibition of TTLL6 function, that tubulin polyglutamylation was essential for maintaining the structural integrity of the ciliary axonemes. The results suggest an association of the dysregulation of tubulin polyglutamylation with a ciliary disease, Joubert syndrome. We also demonstrated that appropriate polyglutamylation level was essential for the maintenance of retinal photoreceptor cells. In addition, we found that intracellular distribution of a protein in photoreceptor cells were affected by the dysregulated hyper-polyglutamylation in CCP1-deficient mouse retina.
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Research Products
(28 results)
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[Journal Article] CEP41 is mutated in Joubert syndrome and is required for tubulin glutamylation at the cilium2012
Author(s)
Lee JE, Silhavy JL, Zaki MS, Schroth J, Bielas SL, Marsh SE, Olvera J, Brancati F, Iannicelli M, Ikegami K, 他20名
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Journal Title
Nat Genet
Volume: 44
Pages: 193-199
DOI
Peer Reviewed
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