- Article
BBS Proteins Affect Ciliogenesis and Are Essential for Hedgehog Signaling, but Not for Formation of iPSC-Derived RPE-65 Expressing RPE-Like Cells
- Caroline Amalie Brunbjerg Hey,
- Lasse Jonsgaard Larsen,
- Zeynep Tümer,
- Karen Brøndum-Nielsen,
- Karen Grønskov,
- Tina Duelund Hjortshøj and
- Lisbeth Birk Møller
Bardet-Biedl syndrome (BBS) is a ciliopathy characterized by retinal dystrophy, renal cysts, obesity and polydactyly. BBS genes have been implicated in ciliogenesis, hedgehog signaling and retinal pigment epithelium maturation. BBS1 and BBS5 are memb...