Mechanism of renal cyst formation induced by abnormal signaling through basal body

2021 Fiscal Year Final Research Report

• Project/Area Number: 18K15983
• Research Category: Grant-in-Aid for Early-Career Scientists
• Allocation Type: Multi-year Fund
• Review Section: Basic Section 53040:Nephrology-related
• Research Institution: Kyoto Prefectural University of Medicine
• Principal Investigator: MATSUO KAZUHIKO 京都府立医科大学, 医学(系)研究科(研究院), 助教 (70599753)
• Project Period (FY): 2018-04-01 – 2022-03-31
• Keywords: 中心小体 / 一次繊毛

Outline of Final Research Achievements

The molecular mechanism of renal cyst formation induced by abnormalities in primary cilia is unknown. This study was conducted to elucidate the molecular mechanism by which cystic kidneys are formed by suppression of gene expression of kendrin, which is localized at the base of primary cilia (outside of primary cilia). The applicant has clarified the interaction of kendrin with proteins localized to INV-C in the primary cilia. Analysis of kendrin interaction proteins identified a novel ciliary protein. Furthermore, we found that genetic disruption of kendrin causes abnormal localization of the protein in the primary cilia.

Free Research Field

細胞生物学

Academic Significance and Societal Importance of the Research Achievements

本研究より、一次繊毛外（基底小体）に局在するkendrinが、一次線毛内のタンパク質の局在に寄与することで一次繊毛を介したシグナル伝達を調整している可能性が強く示唆される結果を得た。これは、嚢胞腎形成メカニズム解明へのマイルストーンとして意義深いと考えられる。悪化した腎機能を回復させることは困難であるが、本研究の成果をもとに嚢胞腎形成のメカニズム解明が進めば、病態の進行を防ぎ腎機能を悪化させない予防薬の開発や、新規治療薬の開発の一助となる事が期待できる。