[English] 日本語
![](img/lk-miru.gif)
- EMDB-33472: Structure of ATP7B C983S/C985S/D1027A mutant in presence of ATOX1 -
+
Open data
-
Basic information
Entry | ![]() | |||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Title | Structure of ATP7B C983S/C985S/D1027A mutant in presence of ATOX1 | |||||||||||||||
![]() | ||||||||||||||||
![]() |
| |||||||||||||||
![]() | P-type Cu(+) transporter / Translocase | |||||||||||||||
Function / homology | ![]() protein maturation by copper ion transfer / P-type divalent copper transporter activity / copper ion transmembrane transporter activity / P-type monovalent copper transporter activity / P-type Cu+ transporter / copper ion export / sequestering of calcium ion / copper ion import / copper ion transport / xenobiotic detoxification by transmembrane export across the plasma membrane ...protein maturation by copper ion transfer / P-type divalent copper transporter activity / copper ion transmembrane transporter activity / P-type monovalent copper transporter activity / P-type Cu+ transporter / copper ion export / sequestering of calcium ion / copper ion import / copper ion transport / xenobiotic detoxification by transmembrane export across the plasma membrane / intracellular zinc ion homeostasis / response to copper ion / Ion transport by P-type ATPases / intracellular copper ion homeostasis / monoatomic ion transmembrane transport / lactation / trans-Golgi network membrane / establishment of localization in cell / late endosome / copper ion binding / Golgi membrane / Golgi apparatus / ATP hydrolysis activity / mitochondrion / ATP binding / membrane / plasma membrane Similarity search - Function | |||||||||||||||
Biological species | ![]() | |||||||||||||||
Method | single particle reconstruction / cryo EM / Resolution: 3.3 Å | |||||||||||||||
![]() | Yang G / Xu L / Guo J / Wu Z | |||||||||||||||
Funding support | ![]()
| |||||||||||||||
![]() | ![]() Title: Structures of the human Wilson disease copper transporter ATP7B. Authors: Guo-Min Yang / Lingyi Xu / Rou-Min Wang / Xin Tao / Zi-Wei Zheng / Shenghai Chang / Demin Ma / Cheng Zhao / Yi Dong / Shan Wu / Jiangtao Guo / Zhi-Ying Wu / ![]() Abstract: The P-type ATPase ATP7B exports cytosolic copper and plays an essential role in the regulation of cellular copper homeostasis. Mutants of ATP7B cause Wilson disease (WD), an autosomal recessive ...The P-type ATPase ATP7B exports cytosolic copper and plays an essential role in the regulation of cellular copper homeostasis. Mutants of ATP7B cause Wilson disease (WD), an autosomal recessive disorder of copper metabolism. Here, we present cryoelectron microscopy (cryo-EM) structures of human ATP7B in the E1 state in the apo, the putative copper-bound, and the putative cisplatin-bound forms. In ATP7B, the N-terminal sixth metal-binding domain (MBD6) binds at the cytosolic copper entry site of the transmembrane domain (TMD), facilitating the delivery of copper from the MBD6 to the TMD. The sulfur-containing residues in the TMD of ATP7B mark the copper transport pathway. By comparing structures of the E1 state human ATP7B and E2-P state frog ATP7B, we propose the ATP-driving copper transport model of ATP7B. These structures not only advance our understanding of the mechanisms of ATP7B-mediated copper export but can also guide the development of therapeutics for the treatment of WD. | |||||||||||||||
History |
|
-
Structure visualization
Supplemental images |
---|
-
Downloads & links
-EMDB archive
Map data | ![]() | 1.8 MB | ![]() | |
---|---|---|---|---|
Header (meta data) | ![]() ![]() | 15.2 KB 15.2 KB | Display Display | ![]() |
Images | ![]() | 62.1 KB | ||
Filedesc metadata | ![]() | 6.2 KB | ||
Others | ![]() ![]() | 27.2 MB 27.2 MB | ||
Archive directory | ![]() ![]() | HTTPS FTP |
-Related structure data
Related structure data | ![]() 7xukMC ![]() 7xumC ![]() 7xunC ![]() 7xuoC ![]() 8ioyC M: atomic model generated by this map C: citing same article ( |
---|---|
Similar structure data | Similarity search - Function & homology ![]() |
-
Links
EMDB pages | ![]() ![]() |
---|---|
Related items in Molecule of the Month |
-
Map
File | ![]() | ||||||||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Voxel size | X=Y=Z: 1.014 Å | ||||||||||||||||||||
Density |
| ||||||||||||||||||||
Symmetry | Space group: 1 | ||||||||||||||||||||
Details | EMDB XML:
|
-Supplemental data
-Half map: #2
File | emd_33472_half_map_1.map | ||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Projections & Slices |
| ||||||||||||
Density Histograms |
-Half map: #1
File | emd_33472_half_map_2.map | ||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Projections & Slices |
| ||||||||||||
Density Histograms |
-
Sample components
-Entire : ATP7B
Entire | Name: ATP7B |
---|---|
Components |
|
-Supramolecule #1: ATP7B
Supramolecule | Name: ATP7B / type: organelle_or_cellular_component / ID: 1 / Parent: 0 / Macromolecule list: all |
---|---|
Source (natural) | Organism: ![]() |
-Macromolecule #1: Copper-transporting ATPase 2
Macromolecule | Name: Copper-transporting ATPase 2 / type: protein_or_peptide / ID: 1 / Number of copies: 1 / Enantiomer: LEVO / EC number: P-type Cu+ transporter |
---|---|
Source (natural) | Organism: ![]() |
Molecular weight | Theoretical: 162.033516 KDa |
Recombinant expression | Organism: ![]() |
Sequence | String: MPEQERQITA REGASRKILS KLSLPTRAWE PAMKKSFAFD NVGYEGGLDG LGPSSQVATS TVRILGMTCQ SCVKSIEDRI SNLKGIISM KVSLEQGSAT VKYVPSVVCL QQVCHQIGDM GFEASIAEGK AASWPSRSLP AQEAVVKLRV EGMTCQSCVS S IEGKVRKL ...String: MPEQERQITA REGASRKILS KLSLPTRAWE PAMKKSFAFD NVGYEGGLDG LGPSSQVATS TVRILGMTCQ SCVKSIEDRI SNLKGIISM KVSLEQGSAT VKYVPSVVCL QQVCHQIGDM GFEASIAEGK AASWPSRSLP AQEAVVKLRV EGMTCQSCVS S IEGKVRKL QGVVRVKVSL SNQEAVITYQ PYLIQPEDLR DHVNDMGFEA AIKSKVAPLS LGPIDIERLQ STNPKRPLSS AN QNFNNSE TLGHQGSHVV TLQLRIDGMH CKSCVLNIEE NIGQLLGVQS IQVSLENKTA QVKYDPSCTS PVALQRAIEA LPP GNFKVS LPDGAEGSGT DHRSSSSHSP GSPPRNQVQG TCSTTLIAIA GMTCASCVHS IEGMISQLEG VQQISVSLAE GTAT VLYNP SVISPEELRA AIEDMGFEAS VVSESCSTNP LGNHSAGNSM VQTTDGTPTS VQEVAPHTGR LPANHAPDIL AKSPQ STRA VAPQKCFLQI KGMTCASCVS NIERNLQKEA GVLSVLVALM AGKAEIKYDP EVIQPLEIAQ FIQDLGFEAA VMEDYA GSD GNIELTITGM TCASCVHNIE SKLTRTNGIT YASVALATSK ALVKFDPEII GPRDIIKIIE EIGFHASLAQ RNPNAHH LD HKMEIKQWKK SFLCSLVFGI PVMALMIYML IPSNEPHQSM VLDHNIIPGL SILNLIFFIL CTFVQLLGGW YFYVQAYK S LRHRSANMDV LIVLATSIAY VYSLVILVVA VAEKAERSPV TFFDTPPMLF VFIALGRWLE HLAKSKTSEA LAKLMSLQA TEATVVTLGE DNLIIREEQV PMELVQRGDI VKVVPGGKFP VDGKVLEGNT MADESLITGE AMPVTKKPGS TVIAGSINAH GSVLIKATH VGNDTTLAQI VKLVEEAQMS KAPIQQLADR FSGYFVPFII IMSTLTLVVW IVIGFIDFGV VQRYFPNPNK H ISQTEVII RFAFQTSITV LCIASPSSLG LATPTAVMVG TGVAAQNGIL IKGGKPLEMA HKIKTVMFAK TGTITHGVPR VM RVLLLGD VATLPLRKVL AVVGTAEASS EHPLGVAVTK YCKEELGTET LGYCTDFQAV PGCGIGCKVS NVEGILAHSE RPL SAPASH LNEAGSLPAE KDAVPQTFSV LIGNREWLRR NGLTISSDVS DAMTDHEMKG QTAILVAIDG VLCGMIAIAD AVKQ EAALA VHTLQSMGVD VVLITGDNRK TARAIATQVG INKVFAEVLP SHKVAKVQEL QNKGKKVAMV GDGVNDSPAL AQADM GVAI GTGTDVAIEA ADVVLIRNDL LDVVASIHLS KRTVRRIRIN LVLALIYNLV GIPIAAGVFM PIGIVLQPWM GSAAMA ASS VSVVLSSLQL KCYKKPDLER YEAQAHGHMK PLTASQVSVH IGMDDRWRDS PRATPWDQVS YVSQVSLSSL TSDKPSR HS AAADDDGDKW SLLLNGRDEE QYIVDELTSR GRDYKDDDDK WSHPQFEKGG GGSGGSAWSH PQFEK UniProtKB: Copper-transporting ATPase 2 |
-Experimental details
-Structure determination
Method | cryo EM |
---|---|
![]() | single particle reconstruction |
Aggregation state | particle |
-
Sample preparation
Buffer | pH: 7.4 |
---|---|
Vitrification | Cryogen name: ETHANE |
-
Electron microscopy
Microscope | FEI TITAN KRIOS |
---|---|
Image recording | Film or detector model: GATAN K2 SUMMIT (4k x 4k) / Average electron dose: 62.0 e/Å2 |
Electron beam | Acceleration voltage: 300 kV / Electron source: ![]() |
Electron optics | Illumination mode: OTHER / Imaging mode: BRIGHT FIELD / Nominal defocus max: 1.3 µm / Nominal defocus min: 1.1 µm |
Experimental equipment | ![]() Model: Titan Krios / Image courtesy: FEI Company |
-
Image processing
Startup model | Type of model: OTHER |
---|---|
Final reconstruction | Resolution.type: BY AUTHOR / Resolution: 3.3 Å / Resolution method: FSC 0.143 CUT-OFF / Number images used: 403645 |
Initial angle assignment | Type: OTHER |
Final angle assignment | Type: OTHER |