Dataset Information

CEP19-RABL2 GTPase complex in ciliary transport

ABSTRACT: The mechanisms of how highly conserved intraflagellar transport (IFT) protein complexes direct ciliary entry remain unknown. Exploring human ciliopathy genes, including monogenic obesity syndrome genes, has been a rich source of critical ciliary mechanisms. Using AP-MS purification of interactors of the CEP19 monogenic obesity gene, we identify factors and the first known mechanism for triggering ciliary entry of IFT complexes. CEP19 binds the CEP350 and FOP centriolar components and the highly conserved RABL2 GTPase. We discovered that the CEP19-RABL2 complex is first recruited by the centriolar CEP350/FOP complex, concurrently activates nucleotide exchange to capture and release intraflagellar transport B holocomplexes from a large centriolar pool to intraflagellar transport. The CEP19 network of interacting proteins regulates ciliary entry and ciliation, providing a new example of ciliary mechanisms deficient in monogenic obesity syndromes.

INSTRUMENT(S): LTQ Orbitrap

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Retinal Pigment Epithelial Cell, Retinal Pigment Epithelium Cell

SUBMITTER: Janos Demeter

LAB HEAD: Peter K. Jackson

PROVIDER: PXD006757 | Pride | 2017-06-21

REPOSITORIES: Pride

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Publications

The CEP19-RABL2 GTPase Complex Binds IFT-B to Initiate Intraflagellar Transport at the Ciliary Base.

Kanie Tomoharu T Abbott Keene Louis KL Mooney Nancie Ann NA Plowey Edward Douglas ED Demeter Janos J Jackson Peter Kent PK

Developmental cell 20170615 1

Highly conserved intraflagellar transport (IFT) protein complexes direct both the assembly of primary cilia and the trafficking of signaling molecules. IFT complexes initially accumulate at the base of the cilium and periodically enter the cilium, suggesting an as-yet-unidentified mechanism that triggers ciliary entry of IFT complexes. Using affinity-purification and mass spectrometry of interactors of the centrosomal and ciliopathy protein, CEP19, we identify CEP350, FOP, and the RABL2B GTPase ...[more]

PMID: 28625565

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Dataset Information

CEP19-RABL2 GTPase complex in ciliary transport

Publications

The CEP19-RABL2 GTPase Complex Binds IFT-B to Initiate Intraflagellar Transport at the Ciliary Base.

Similar Datasets

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