PXD006757-1

PXD006757 is an original dataset announced via ProteomeXchange.

Title	CEP19-RABL2 GTPase complex in ciliary transport
Description	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.
HostingRepository	PRIDE
AnnounceDate	2017-06-21
AnnouncementXML	Submission_2017-06-21_06:08:38.xml
DigitalObjectIdentifier	https://dx.doi.org/10.6019/PXD006757
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Supported dataset by repository
PrimarySubmitter	Janos Demeter
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	Phospho; Propionamide; Glu->pyro-Glu; Deamidated; Methyl; Oxidation; Acetyl; GlyGly; Gln->pyro-Glu
Instrument	LTQ Orbitrap

Revision	Datetime	Status	ChangeLog Entry
0	2017-06-20 03:35:27	ID requested
⏵ 1	2017-06-21 06:08:40	announced

Kanie T, Abbott KL, Mooney NA, Plowey ED, Demeter J, Jackson PK, The CEP19-RABL2 GTPase Complex Binds IFT-B to Initiate Intraflagellar Transport at the Ciliary Base. Dev Cell, 42(1):22-36.e12(2017) [pubmed]

curator keyword: Biological

submitter keyword: Cilia, ciliopathy, CEP19-RABL2B complex, Human Genetics, Genetics", obesity, centrosome, centriole, GTPase, proteomics, metabolic syndrome, Bardet-Biedl syndrome, intraflagellar transport, microtubules

Peter K. Jackson
contact affiliation	Baxter Laboratory for Stem Cell Biology Department of Microbiology & Immunology Stanford University School of Medicine 269 Campus Drive CCSR 3205b Stanford, CA 94305-5175
contact email	pjackson@stanford.edu
lab head
Janos Demeter
contact affiliation	Stanford University
contact email	jdemeter@stanford.edu
dataset submitter

Dataset FTP location NOTE: Most web browsers have now discontinued native support for FTP access within the browser window. But you can usually install another FTP app (we recommend FileZilla) and configure your browser to launch the external application when you click on this FTP link. Or otherwise, launch an app that supports FTP (like FileZilla) and use this address: ftp://ftp.pride.ebi.ac.uk/pride/data/archive/2017/06/PXD006757

PRIDE project URI

• PRIDE
  1. PXD006757
     1. Label: PRIDE project
     2. Name: CEP19-RABL2 GTPase complex in ciliary transport