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Article

The Bardet-Biedl syndrome protein complex is an adapter expanding the cargo range of intraflagellar transport trains for ciliary export.

Proceedings of the National Academy of Sciences of the United States of America - 30 Jan 2018

Liu Peiwei, Lechtreck Karl F

Abstract excerpt

Bardet-Biedl syndrome (BBS) is a ciliopathy resulting from defects in the BBSome, a conserved protein complex. BBSome mutations affect ciliary membrane composition, impairing cilia-based signaling. The mechanism by which the BBSome regulates ciliary membrane content remains unknown. Chlamydomonas bbs mutants lack phototaxis and accumulate phospholipase D (PLD) in the ciliary membrane. Single particle imaging...

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Identifiers

PMID: 29339469
DOI: 10.1073/pnas.1713226115
PMCID: PMC5798339

Genes

BBS4

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

The Bardet-Biedl syndrome protein complex is an adapter expanding the cargo range of intraflagellar transport trains for ciliary export.PMID 29339469Read ↗

Chlamydomonas LZTFL1 mediates phototaxis via controlling BBSome recruitment to the basal body and its reassembly at the ciliary tipSemantic proximity Read ↗

The <i>Chlamydomonas reinhardtii</i> BBSome is an IFT cargo required for export of specific signaling proteins from flagellaSemantic proximity Read ↗

The BBSome assembly is spatially controlled by BBS1 and BBS4 in human cells.Semantic proximity Read ↗

Ciliary protein trafficking mediated by IFT and BBSome complexes with the aid of kinesin-2 and dynein-2 motorsSemantic proximity Read ↗

The BBSome assembly is spatially controlled by BBS1 and BBS4 in human cellsSemantic proximity Read ↗

Bardet-Biedl Syndrome 1 Mutations Differentially Impact BBSome Integrity and its Function in Ciliary TraffickingSemantic proximity Read ↗

Bardet-Biedl syndrome 1 mutations differentially impact BBSome integrity and ciliary trafficking.Semantic proximity Read ↗

Two-step process regulating the ciliary entrance of non-ciliary proteins: BBSome functions in the first step, upstream of the ciliary gateSemantic proximity Read ↗

Cycling of the signaling protein phospholipase D through cilia requires the BBSome only for the export phase.Semantic proximity Read ↗

Unraveling the Intricate Cargo-BBSome Coupling Mechanism at the Ciliary TipSemantic proximity Read ↗

IFT27, encoding a small GTPase component of IFT particles, is mutated in a consanguineous family with Bardet-Biedl syndrome.Semantic proximity Read ↗

ARL3 mediates BBSome ciliary turnover by promoting its outward movement across the transition zoneSemantic proximity Read ↗

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