+
Open data
-
Basic information
| Entry | Database: PDB / ID: 7q3d | ||||||
|---|---|---|---|---|---|---|---|
| Title | Structure of the human CPLANE complex | ||||||
Components |
| ||||||
Keywords | PROTEIN TRANSPORT / ciliogenesis / ciliopathies / longin / lipid binding | ||||||
| Function / homology | Function and homology informationaxonemal basal plate / negative regulation of neural crest formation / negative regulation of fibroblast growth factor receptor signaling pathway involved in neural plate anterior/posterior pattern formation / tongue morphogenesis / respiratory system development / regulation of embryonic cell shape / digestive system development / septin cytoskeleton organization / embryonic body morphogenesis / regulation of ruffle assembly ...axonemal basal plate / negative regulation of neural crest formation / negative regulation of fibroblast growth factor receptor signaling pathway involved in neural plate anterior/posterior pattern formation / tongue morphogenesis / respiratory system development / regulation of embryonic cell shape / digestive system development / septin cytoskeleton organization / embryonic body morphogenesis / regulation of ruffle assembly / podocyte cell migration / regulation of fibroblast migration / spinal cord dorsal/ventral patterning / establishment of planar polarity / protein localization to organelle / intraciliary transport / regulation of cilium assembly / auditory receptor cell morphogenesis / ciliary transition zone / embryonic skeletal system morphogenesis / motile cilium assembly / cilium organization / negative regulation of cell division / positive regulation of cilium assembly / camera-type eye development / non-motile cilium assembly / regulation of smoothened signaling pathway / positive regulation of smoothened signaling pathway / circulatory system development / limb development / neural tube development / regulation of establishment of cell polarity / regulation of focal adhesion assembly / motile cilium / embryonic digit morphogenesis / hair follicle morphogenesis / smoothened signaling pathway / roof of mouth development / axoneme / regulation of ossification / cilium assembly / hair follicle development / negative regulation of keratinocyte proliferation / Hedgehog 'off' state / keratinocyte differentiation / vesicle-mediated transport / centriole / phosphatidylinositol binding / negative regulation of cell migration / neural tube closure / kidney development / establishment of protein localization / negative regulation of canonical Wnt signaling pathway / nervous system development / protein transport / regulation of protein localization / cytoskeleton / cilium / ciliary basal body / negative regulation of cell population proliferation / cell division / intracellular membrane-bounded organelle / cell surface / extracellular exosome / plasma membrane / cytoplasm / cytosol 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
| ||||||
Citation | Journal: Sci Adv / Year: 2022Title: 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 |
|
-
Structure visualization
| Structure viewer | Molecule: Molmil Jmol/JSmol |
|---|
-
Downloads & links
-
Download
| PDBx/mmCIF format | 7q3d.cif.gz | 547.5 KB | Display | PDBx/mmCIF format |
|---|---|---|---|---|
| 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 |
|---|---|---|---|---|
| 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 ( |
|---|---|
| Similar structure data | Similarity search - Function & homology F&H Search |
-
Links
-
Assembly
| Deposited unit | ![]()
|
|---|---|
| 1 |
|
-
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: ![]() |
|---|---|
| #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: ![]() |
| #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: ![]() |
-Experimental details
-Experiment
| Experiment | Method: ELECTRON MICROSCOPY |
|---|---|
| EM experiment | Aggregation state: PARTICLE / 3D reconstruction method: single particle reconstruction |
-
Sample preparation
| Component | Name: CPLANE / Type: COMPLEX / Entity ID: all / Source: RECOMBINANT |
|---|---|
| Source (natural) | Organism: Homo sapiens (human) |
| Source (recombinant) | Organism: ![]() |
| 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 |
|---|---|
| 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 |
| |||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 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 |
Movie
Controller
About Yorodumi




Homo sapiens (human)
Citation




PDBj




FIELD EMISSION GUN