Clarification of the pathogenesis of cyst formation in autosomal dominant polycystic kidney disease using a TRPP2 697fsX transgenic mouse model

2014 Fiscal Year Final Research Report

• Project/Area Number: 23790936
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Kidney internal medicine
• Research Institution: Kanazawa University
• Principal Investigator: YAMADA Kazunori 金沢大学, 医薬保健学総合研究科, 特任准教授 (90397224)
• Project Period (FY): 2011-04-28 – 2015-03-31
• Keywords: 常染色体優性多発性嚢胞腎 / モデルマウス

Outline of Final Research Achievements

Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary disorder characterized by multiple cysts in both kidneys and other organs. We obtained TRPP2 697fsX, which causes the aberrant localization of polycystin-2, from a patient with ADPKD and established TRPP2 697fsX transgenic mice. These mice exhibited proteinuria, renal insufficiency at 4 weeks, and cyst formation at a minimum age of 6 months. These data indicate that TRPP2 697fsX transgenic mice can be used as mice models of ADPKD. Hence, using these models, we will further analyze the mechanism underlying cyst formation in ADPKD.

Free Research Field

腎臓内科