Abstract
The structures of the bovine and human BBSome reveal that a conformational change is required to recruit the complex to the ciliary membrane.
Highlights
Bardet-Biedl syndrome is a genetic disease that causes blindness, obesity and kidney anomalies (Brown and Witman, 2014; Sheffield, 2010)
It arises due to mutations in more than 20 genes, eight of which encode the proteins that form a large complex known as the BBSome (Nachury et al, 2007). This complex is found in the cilia that protrude from most eukaryotic cells and have important roles in motility and signaling (Wingfield et al, 2018). It is in charge of exporting membrane proteins from the cilium to the cell body through a process known as intraflagellar transport (Lechtreck et al, 2009; Figure 1)
The high resolution of the newly reported structures allowed the accurate visualization of amino acid side-chains in the complex, something that was not possible in a medium-resolution structure that had been published previously (Chou et al, 2019)
Summary
Bardet-Biedl syndrome is a genetic disease that causes blindness, obesity and kidney anomalies (Brown and Witman, 2014; Sheffield, 2010). It is in charge of exporting membrane proteins from the cilium to the cell body through a process known as intraflagellar transport (Lechtreck et al, 2009; Figure 1). The details of how the BBSome proteins organize into a complex that can recognize signaling molecules in the cilium membrane for intraflagellar transport are not fully understood.
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