The C-terminal tail of polycystin-1 suppresses cystic disease in a mitochondrial enzyme-dependent fashion

oleh: Laura Onuchic, Valeria Padovano, Giorgia Schena, Vanathy Rajendran, Ke Dong, Xiaojian Shi, Raj Pandya, Victoria Rai, Nikolay P. Gresko, Omair Ahmed, TuKiet T. Lam, Weiwei Wang, Hongying Shen, Stefan Somlo, Michael J. Caplan

Format: Article
Diterbitkan: Nature Portfolio 2023-03-01

Deskripsi

Mutations in the gene encoding PC1 cause ADPKD, a common genetic renal disease. Here, the authors show that expression of the C-terminal 200 amino acids of the large PC1 protein in mouse models of ADPKD suppresses cystic disease through an interaction with the mitochondrial enzyme NNT.