The conserved Bardet-Biedl Syndrome proteins assemble a coat that traffics membrane proteins to cilia

By Hua Jin, Susan Roehl White, Toshinobu Shida, S. Schulz, Mike Aguiar, Steven P. Gygi, J. Bazan, M. Nachury. Cell, 2010

627 citations recorded by the source corpus.

Summary The BBSome is a complex of Bardet-Biedl Syndrome (BBS) proteins that shares common structural elements with COPI, COPII and clathrin coats. Here we show that the BBSome constitutes a coat complex that sorts membrane proteins to primary cilia. Biochemically, the BBSome is the major effector of the Arf-like GTPase Arl6/ BBS3. In vivo, the BBSome and Arl6 localize to ciliary punctae and Arl6GTP is required to target the BBSome to cilia. Congruently, GTP-bound Arl6 and acidic phospholipids are sufficient to efficiently recruit the BBSome to chemically defined liposomes. Finally, ultrastructural analyses demonstrate that BBSome binding to liposomes produces distinct patches of polymerized coat. Since we establish that the ciliary targeting signal of somatostatin receptor 3 needs to be directly recognized by the BBSome to mediate targeting to cilia, we propose that trafficking to cilia entails the coupling of BBSome coat polymerization to the recognition of sorting signals.

Subfields: Genetics, Molecular Biology

View Paper: https://www.semanticscholar.org/paper/fd2204c3d3ecbe4858e3a91542fad2f7add8d93e

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In Genetics

1. N. Katsanis
2. Arturo Alvarez-Buylla

In Molecular Biology

1. Jose Manuel García-Verdugo
2. Gokul Ramaswami
3. N. Katsanis
4. Arturo Alvarez-Buylla

Citations in the corpus

• Arf Family G Proteins and their regulators: roles in membrane transport, development and disease (2011, background): “ARL6 is a BBS subunit (BBS3) and is required in its GTP-bound form to recruit the BBSome onto the plasma membrane to drive cargo sorting into cili”
• Non‐syndromic retinitis pigmentosa (2018, neutral): “, 2011); however, four of the genes that encode BBSome subunits (BBS1, BBS2, BBS9, and TTC8), as well as the gene that encodes ARL6 (a protein that recruits the BBSome complex to the membrane), are associated with non-syndromic RP (Abu-Safieh et al., 2012; Estrada-Cuzcano et al., 2012a; Goyal et al.”
• Ciliogenesis: building the cell's antenna (2011, background): “The BBSome also interacts with ARF-like 6 (Arl6; also known as BBS3), a GTPase, to form a coat complex that sorts membrane proteins, such as SSTR3, to cili”
• A Transition Zone Complex Regulates Mammalian Ciliogenesis and Ciliary Membrane Composition (2011, background): “Other ciliary localization sequences, such as that of Sstr3, bind the BBSome, a protein complex involved in BB”