BBSome-mediated cargo-targeting to cilium

Stable Identifier
R-HSA-5620922
Type
Pathway
Species
Homo sapiens
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The BBSome is a stable complex consisting of 7 Bardet-Biedl proteins (BBS1, 2, 4, 5, 7, 8 and 9) and BBIP10 that has roles in promoting IFT and trafficking proteins to the cilum (Blacque et al, 2004; Nachury et al, 2007; Loktev et al, 2008; Jin et al, 2010; reviewed in Sung and Leroux 2013). The BBSome is the primary effector of ARL6/BBS3, a small GTPase that binds the BBSome in complex with associated membrane proteins that are destined for the ciliary membrane (Jin et al, 2010; Nachury et al, 2007; Zhang et al, 2011; Seo et al, 2011). Components of the BBSome are enriched in TPR and beta-propeller motifs and are thought to form a linear coat on membranes that functions with ARL6 to target proteins to the cilium (Jin et al, 2010; reviewed in Nachury et al, 2010).

Literature References
PubMed ID Title Journal Year
20603001 The conserved Bardet-Biedl syndrome proteins assemble a coat that traffics membrane proteins to cilia

Jin, H, White, SR, Shida, T, Schulz, S, Aguiar, M, Gygi, SP, Bazan, JF, Nachury, MV

Cell 2010
15231740 Loss of C. elegans BBS-7 and BBS-8 protein function results in cilia defects and compromised intraflagellar transport

Blacque, OE, Reardon, MJ, Li, C, McCarthy, J, Mahjoub, MR, Ansley, SJ, Badano, JL, Mah, AK, Beales, PL, Davidson, WS, Johnsen, RC, Audeh, M, Plasterk, RH, Baillie, DL, Katsanis, N, Quarmby, LM, Wicks, SR, Leroux, MR

Genes Dev. 2004
22139371 Bardet-Biedl syndrome 3 (Bbs3) knockout mouse model reveals common BBS-associated phenotypes and Bbs3 unique phenotypes

Zhang, Q, Nishimura, D, Seo, S, Vogel, T, Morgan, DA, Searby, C, Bugge, K, Stone, EM, Rahmouni, K, Sheffield, VC

Proc. Natl. Acad. Sci. U.S.A. 2011
22072986 A novel protein LZTFL1 regulates ciliary trafficking of the BBSome and Smoothened

Seo, S, Zhang, Q, Bugge, K, Breslow, DK, Searby, CC, Nachury, MV, Sheffield, VC

PLoS Genet. 2011
24296415 The roles of evolutionarily conserved functional modules in cilia-related trafficking

Sung, CH, Leroux, MR

Nat. Cell Biol. 2013
19575670 Trafficking to the ciliary membrane: how to get across the periciliary diffusion barrier?

Nachury, MV, Seeley, ES, Jin, H

Annu. Rev. Cell Dev. Biol. 2010
17574030 A core complex of BBS proteins cooperates with the GTPase Rab8 to promote ciliary membrane biogenesis

Nachury, MV, Loktev, AV, Zhang, Q, Westlake, CJ, Peränen, J, Merdes, A, Slusarski, DC, Scheller, RH, Bazan, JF, Sheffield, VC, Jackson, PK

Cell 2007
19081074 A BBSome subunit links ciliogenesis, microtubule stability, and acetylation

Loktev, AV, Zhang, Q, Beck, JS, Searby, CC, Scheetz, TE, Bazan, JF, Slusarski, DC, Sheffield, VC, Jackson, PK, Nachury, MV

Dev. Cell 2008
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