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Investigating the genetic interaction between Pkhd1 and Pkd1

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干细胞与再生医学数据中心2022-02-20 更新2024-03-06 收录
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http://data.iscr.ac.cn/Article?id=8bb350a8eff96f3d53b56910e67e464e
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The lack of rapidly progressive murine models reflecting the classical infantile disease in ARPKD inhibits progress to understanding pathogenesis. The role that primary cilia play in PKD is also controversial. Here new mouse and rat models, generated by interbreeding appropriate ARPKD and ADPKD strains are characterized; better reflecting the severe human disease. Analysis of mRNA expression in the individual ARPKD and ADPKD models, and the combined mice highlighted different disrupted pathways but with a commonality of dysregulated mechanisms associated with primary cilia. These models will help understand ARPKD and improve preclinical testing for this disease. The study also, in unbiased way, reinforces that cilia are important for pathogenesis in both disorders.
提供机构:
Mayo Clinic
创建时间:
2022-02-20
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