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Structural basis for membrane targeting of the BBSome by ARL6.

MPG-Autoren
http://pubman.mpdl.mpg.de/cone/persons/resource/persons138063

Mourao,  Andre
Lorentzen, Esben / Intraflagellar Transport, Max Planck Institute of Biochemistry, Max Planck Society;

http://pubman.mpdl.mpg.de/cone/persons/resource/persons78333

Lorentzen,  Esben
Lorentzen, Esben / Intraflagellar Transport, Max Planck Institute of Biochemistry, Max Planck Society;

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Zitation

Mourao, A., Nager, A. R., Nachury, M. V., & Lorentzen, E. (2014). Structural basis for membrane targeting of the BBSome by ARL6. Nature structural & molecular biology, 21(12), 1035-1041. doi:10.1038/nsmb.2920.


Zitierlink: http://hdl.handle.net/11858/00-001M-0000-0024-61C7-0
Zusammenfassung
The BBSome is a coat-like ciliary trafficking complex composed of proteins mutated in Bardet-Biedl syndrome (BBS). A critical step in BBSome-mediated sorting is recruitment of the BBSome to membranes by the GTP-bound Arf-like GTPase ARL6. We have determined crystal structures of Chlamydomonas reinhardtii ARL6-GDP, ARL6-GTP and the ARL6-GTP-BBS1 complex. The structures demonstrate how ARL6-GTP binds the BBS1 beta-propeller at blades 1 and 7 and explain why GTP- but not GDP-bound ARL6 can recruit the BBSome to membranes. Single point mutations in the ARL6-GTP-BBS1 interface abolish the interaction of ARL6 with the BBSome and prevent the import of BBSomes into cilia. Furthermore, we show that BBS1 with the M390R mutation, responsible for 30% of all reported BBS disease cases, fails to interact with ARL6-GTP, thus providing a molecular rationale for patient pathologies.