+Open data
-Basic information
Entry | Database: PDB / ID: 7q3d | ||||||
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Title | Structure of the human CPLANE complex | ||||||
Components |
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Keywords | PROTEIN TRANSPORT / ciliogenesis / ciliopathies / longin / lipid binding | ||||||
Function / homology | Function and homology information negative regulation of neural crest formation / negative regulation of fibroblast growth factor receptor signaling pathway involved in neural plate anterior/posterior pattern formation / axonemal basal plate / respiratory system development / tongue morphogenesis / auditory receptor cell morphogenesis / regulation of embryonic cell shape / septin cytoskeleton organization / digestive system development / embryonic body morphogenesis ...negative regulation of neural crest formation / negative regulation of fibroblast growth factor receptor signaling pathway involved in neural plate anterior/posterior pattern formation / axonemal basal plate / respiratory system development / tongue morphogenesis / auditory receptor cell morphogenesis / regulation of embryonic cell shape / septin cytoskeleton organization / digestive system development / embryonic body morphogenesis / regulation of ruffle assembly / regulation of fibroblast migration / podocyte cell migration / establishment of planar polarity / protein localization to organelle / intraciliary transport / spinal cord dorsal/ventral patterning / ciliary transition zone / regulation of cilium assembly / motile cilium assembly / embryonic skeletal system morphogenesis / cilium organization / negative regulation of cell division / circulatory system development / positive regulation of cilium assembly / non-motile cilium assembly / camera-type eye development / regulation of smoothened signaling pathway / positive regulation of smoothened signaling pathway / motile cilium / limb development / neural tube development / regulation of establishment of cell polarity / regulation of focal adhesion assembly / embryonic digit morphogenesis / hair follicle morphogenesis / smoothened signaling pathway / roof of mouth development / axoneme / hair follicle development / cilium assembly / regulation of ossification / negative regulation of keratinocyte proliferation / Hedgehog 'off' state / vesicle-mediated transport / keratinocyte differentiation / centriole / phosphatidylinositol binding / ciliary basal body / negative regulation of cell migration / kidney development / neural tube closure / establishment of protein localization / negative regulation of canonical Wnt signaling pathway / cilium / protein transport / regulation of protein localization / nervous system development / cytoskeleton / negative regulation of cell population proliferation / cell division / cell surface / extracellular exosome / plasma membrane / cytoplasm Similarity search - Function | ||||||
Biological species | Homo sapiens (human) | ||||||
Method | ELECTRON MICROSCOPY / single particle reconstruction / cryo EM / Resolution: 3.35 Å | ||||||
Authors | Langousis, G. / Cavadini, S. / Kempf, G. / Matthias, P. | ||||||
Funding support | 1items
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Citation | Journal: Sci Adv / Year: 2022 Title: Structure of the ciliogenesis-associated CPLANE complex. Authors: Gerasimos Langousis / Simone Cavadini / Niels Boegholm / Esben Lorentzen / Georg Kempf / Patrick Matthias / Abstract: Dysfunctional cilia cause pleiotropic human diseases termed ciliopathies. These hereditary maladies are often caused by defects in cilia assembly, a complex event that is regulated by the ...Dysfunctional cilia cause pleiotropic human diseases termed ciliopathies. These hereditary maladies are often caused by defects in cilia assembly, a complex event that is regulated by the ciliogenesis and planar polarity effector (CPLANE) proteins Wdpcp, Inturned, and Fuzzy. CPLANE proteins are essential for building the cilium and are mutated in multiple ciliopathies, yet their structure and molecular functions remain elusive. Here, we show that mammalian CPLANE proteins comprise a bona fide complex and report the near-atomic resolution structures of the human Wdpcp-Inturned-Fuzzy complex and of the mouse Wdpcp-Inturned-Fuzzy complex bound to the small guanosine triphosphatase Rsg1. Notably, the crescent-shaped CPLANE complex binds phospholipids such as phosphatidylinositol 3-phosphate via multiple modules and a CPLANE ciliopathy mutant exhibits aberrant lipid binding. Our study provides critical structural and functional insights into an enigmatic ciliogenesis-associated complex as well as unexpected molecular rationales for ciliopathies. | ||||||
History |
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-Structure visualization
Structure viewer | Molecule: MolmilJmol/JSmol |
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-Downloads & links
-Download
PDBx/mmCIF format | 7q3d.cif.gz | 547.5 KB | Display | PDBx/mmCIF format |
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PDB format | pdb7q3d.ent.gz | 440.5 KB | Display | PDB format |
PDBx/mmJSON format | 7q3d.json.gz | Tree view | PDBx/mmJSON format | |
Others | Other downloads |
-Validation report
Summary document | 7q3d_validation.pdf.gz | 1.2 MB | Display | wwPDB validaton report |
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Full document | 7q3d_full_validation.pdf.gz | 1.2 MB | Display | |
Data in XML | 7q3d_validation.xml.gz | 46.6 KB | Display | |
Data in CIF | 7q3d_validation.cif.gz | 70.3 KB | Display | |
Arichive directory | https://data.pdbj.org/pub/pdb/validation_reports/q3/7q3d ftp://data.pdbj.org/pub/pdb/validation_reports/q3/7q3d | HTTPS FTP |
-Related structure data
Related structure data | 13789MC 7q3eC M: map data used to model this data C: citing same article (ref.) |
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Similar structure data | Similarity search - Function & homologyF&H Search |
-Links
-Assembly
Deposited unit |
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1 |
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-Components
#1: Protein | Mass: 89649.320 Da / Num. of mol.: 1 Source method: isolated from a genetically manipulated source Source: (gene. exp.) Homo sapiens (human) / Gene: WDPCP, BBS15, C2orf86, FRITZ / Production host: Spodoptera frugiperda (fall armyworm) / References: UniProt: O95876 |
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#2: Protein | Mass: 108150.633 Da / Num. of mol.: 1 Source method: isolated from a genetically manipulated source Source: (gene. exp.) Homo sapiens (human) / Gene: INTU, KIAA1284, PDZD6, PDZK6 / Production host: Spodoptera frugiperda (fall armyworm) / References: UniProt: Q9ULD6 |
#3: Protein | Mass: 45721.949 Da / Num. of mol.: 1 Source method: isolated from a genetically manipulated source Source: (gene. exp.) Homo sapiens (human) / Gene: FUZ, FY / Production host: Spodoptera frugiperda (fall armyworm) / References: UniProt: Q9BT04 |
-Experimental details
-Experiment
Experiment | Method: ELECTRON MICROSCOPY |
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EM experiment | Aggregation state: PARTICLE / 3D reconstruction method: single particle reconstruction |
-Sample preparation
Component | Name: CPLANE / Type: COMPLEX / Entity ID: all / Source: RECOMBINANT |
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Source (natural) | Organism: Homo sapiens (human) |
Source (recombinant) | Organism: Spodoptera frugiperda (fall armyworm) |
Buffer solution | pH: 8 |
Specimen | Embedding applied: NO / Shadowing applied: NO / Staining applied: NO / Vitrification applied: YES |
Vitrification | Cryogen name: ETHANE |
-Electron microscopy imaging
Experimental equipment | Model: Titan Krios / Image courtesy: FEI Company |
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Microscopy | Model: FEI TITAN KRIOS |
Electron gun | Electron source: FIELD EMISSION GUN / Accelerating voltage: 300 kV / Illumination mode: SPOT SCAN |
Electron lens | Mode: BRIGHT FIELD |
Image recording | Electron dose: 1.04 e/Å2 / Film or detector model: FEI FALCON IV (4k x 4k) |
-Processing
EM software |
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CTF correction | Type: PHASE FLIPPING AND AMPLITUDE CORRECTION | |||||||||||||||||||||
3D reconstruction | Resolution: 3.35 Å / Resolution method: FSC 0.143 CUT-OFF / Num. of particles: 129902 / Symmetry type: POINT | |||||||||||||||||||||
Atomic model building | Protocol: FLEXIBLE FIT / Space: REAL |