WORKPACKAGE 4

The role of BBS, OFD1, and NPHP proteins in Wnt signalling, tubulogenesis and cyst formation

Leaders of the WP are Gerd Walz and Heymut Omran from the Universitaetsklinikum (Freiburg, Germany).

1. This workpackage will analyze the interaction between BBS/OFD1/NPHP and Wnt signalling molecules, and utilize functional in vitro and in vivo assay systems to assess the role of these molecules in canonical and non-canonical Wnt signalling.
2. The zebrafish pronephros model will be utilized to determine the role of BBS/OFD1/NPHP proteins in tubular morphogenesis, oriented cell division, and ciliary motility will be determined.
3. Mouse models deficient for BBS/OFD1/NPHP and selected PCP proteins will be analyzed for abnormal renal development to validate the role of BBS/OFD1/NPHP in Wnt signalling, derived from in vitro experiments and the zebrafish analysis.