Loading
PDBj
MenuPDBj@FacebookPDBj@X(formerly Twitter)PDBj@BlueSkyPDBj@YouTubewwPDB FoundationwwPDBDonate
RCSB PDBPDBeBMRBAdv. SearchSearch help

6VBV

Structure of the bovine BBSome:ARL6:GTP complex

Summary for 6VBV
Entry DOI10.2210/pdb6vbv/pdb
EMDB information21145
DescriptorBardet-Biedl syndrome 18 protein, CALCIUM ION, GUANOSINE-5'-TRIPHOSPHATE, ... (11 entities in total)
Functional Keywordscilia, ciliopathy, complex, membrane-protein transport, protein transport
Biological sourceBos taurus (Bovine)
More
Total number of polymer chains9
Total formula weight508169.76
Authors
Singh, S.K.,Gui, M.,Koh, F.,Yip, M.C.J.,Brown, A. (deposition date: 2019-12-19, release date: 2020-01-29, Last modification date: 2024-03-06)
Primary citationSingh, S.K.,Gui, M.,Koh, F.,Yip, M.C.,Brown, A.
Structure and activation mechanism of the BBSome membrane protein trafficking complex.
Elife, 9:-, 2020
Cited by
PubMed Abstract: Bardet-Biedl syndrome (BBS) is a currently incurable ciliopathy caused by the failure to correctly establish or maintain cilia-dependent signaling pathways. Eight proteins associated with BBS assemble into the BBSome, a key regulator of the ciliary membrane proteome. We report the electron cryomicroscopy (cryo-EM) structures of the native bovine BBSome in inactive and active states at 3.1 and 3.5 Å resolution, respectively. In the active state, the BBSome is bound to an Arf-family GTPase (ARL6/BBS3) that recruits the BBSome to ciliary membranes. ARL6 recognizes a composite binding site formed by BBS1 and BBS7 that is occluded in the inactive state. Activation requires an unexpected swiveling of the β-propeller domain of BBS1, the subunit most frequently implicated in substrate recognition, which widens a central cavity of the BBSome. Structural mapping of disease-causing mutations suggests that pathogenesis results from folding defects and the disruption of autoinhibition and activation.
PubMed: 31939736
DOI: 10.7554/eLife.53322
PDB entries with the same primary citation
Experimental method
ELECTRON MICROSCOPY (3.5 Å)
Structure validation

243083

数据于2025-10-15公开中

PDB statisticsPDBj update infoContact PDBjnumon