Cystin is required for maintaining fibrocystin (FPC) levels and safeguarding proteome integrity in mouse renal epithelial cells A mechanistic connection between the kidney defects in cpk mice and human ARPKDRunning title: Cystin deficiency results in FPC degradation
Y. Zhang, C. Yang, W. Wang, N. Haraffuji, P. Stasiak, D. P. Bell, E. Sztul, L. Caldovic, L. M. Guay-Woodford, Z. Bebok
This study shows cystin deficiency reduces fibrocystin (FPC) levels in mouse renal cells, leading to proteome changes and cyst formation similar to ARPKD.
bioRxiv · Apr 19, 2022Cell Biology