Stefan Somlo and colleagues show that the polycystic liver disease genes Prkcsh and Sec63 are required for the proper biogenesis of polycystin complexes. They further show that a Pkd1 transgene can rescue the cystic phenotype of Prkcsh and Sec63 mutant mice, identifying polycystin-1 as the central determinant of cyst formation.
- Sorin V Fedeles
- Xin Tian
- Stefan Somlo