Article
The BBSome assembly is spatially controlled by BBS1 and BBS4 in human cells.
The Journal of biological chemistry - 16 Oct 2020
Prasai Avishek, Schmidt Cernohorska Marketa, Ruppova Klara, Niederlova Veronika, Andelova Monika, Draber Peter, Stepanek Ondrej, Huranova Martina
Abstract excerpt
Bardet-Biedl syndrome (BBS) is a pleiotropic ciliopathy caused by dysfunction of primary cilia. More than half of BBS patients carry mutations in one of eight genes encoding for subunits of a protein complex, the BBSome, which mediates trafficking of ciliary cargoes. In this study, we elucidated the mechanisms of the BBSome assembly in living cells and how this process is spatially regulated. We generated a large...
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