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6a70

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==Structure of the human PKD1/PKD2 complex==
==Structure of the human PKD1/PKD2 complex==
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<StructureSection load='6a70' size='340' side='right'caption='[[6a70]], [[Resolution|resolution]] 3.60&Aring;' scene=''>
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<SX load='6a70' size='340' side='right' viewer='molstar' caption='[[6a70]], [[Resolution|resolution]] 3.60&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[6a70]] is a 4 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6A70 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6A70 FirstGlance]. <br>
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<table><tr><td colspan='2'>[[6a70]] is a 4 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6A70 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6A70 FirstGlance]. <br>
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</td></tr><tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">PKD2, TRPP2 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN]), PKD1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 3.6&#8491;</td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6a70 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6a70 OCA], [http://pdbe.org/6a70 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6a70 RCSB], [http://www.ebi.ac.uk/pdbsum/6a70 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6a70 ProSAT]</span></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=6a70 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6a70 OCA], [https://pdbe.org/6a70 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6a70 RCSB], [https://www.ebi.ac.uk/pdbsum/6a70 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6a70 ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
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[[http://www.uniprot.org/uniprot/PKD2_HUMAN PKD2_HUMAN]] Defects in PKD2 are the cause of polycystic kidney disease 2 (PKD2) [MIM:[http://omim.org/entry/613095 613095]]. PKD2 is a disorder characterized by progressive formation and enlargement of cysts in both kidneys, typically leading to end-stage renal disease in adult life. Cysts also occurs in the liver and other organs. It represents approximately 15% of the cases of autosomal dominant polycystic kidney disease. PKD2 is clinically milder than PKD1 but it has a deleterious impact on overall life expectancy.<ref>PMID:9326320</ref> <ref>PMID:10541293</ref> <ref>PMID:10411676</ref> <ref>PMID:10835625</ref> <ref>PMID:11968093</ref> <ref>PMID:12707387</ref> <ref>PMID:14993477</ref> <ref>PMID:15772804</ref> <ref>PMID:21115670</ref> [[http://www.uniprot.org/uniprot/PKD1_HUMAN PKD1_HUMAN]] Defects in PKD1 are the cause of polycystic kidney disease 1 (PKD1) [MIM:[http://omim.org/entry/173900 173900]]. PKD1 is characterized by progressive formation and enlargement of cysts in both kidneys, typically leading to end-stage renal disease in adult life. Cysts also occurs in the liver and other organs. Its prevalence is estimated at about 1/1000.<ref>PMID:12482949</ref> <ref>PMID:8554072</ref> <ref>PMID:9199561</ref> <ref>PMID:9285784</ref> <ref>PMID:9259200</ref> <ref>PMID:9521593</ref> <ref>PMID:9921908</ref> <ref>PMID:10364515</ref> <ref>PMID:10577909</ref> <ref>PMID:10987650</ref> <ref>PMID:10647901</ref> <ref>PMID:10200984</ref> <ref>PMID:10854095</ref> <ref>PMID:11216660</ref> <ref>PMID:10923040</ref> <ref>PMID:11058904</ref> <ref>PMID:11012875</ref> <ref>PMID:10729710</ref> <ref>PMID:11115377</ref> <ref>PMID:11571556</ref> <ref>PMID:11316854</ref> <ref>PMID:11558899</ref> <ref>PMID:11691639</ref> <ref>PMID:12220456</ref> <ref>PMID:11857740</ref> <ref>PMID:12007219</ref> <ref>PMID:12070253</ref> <ref>PMID:11967008</ref> <ref>PMID:11773467</ref> <ref>PMID:12842373</ref> <ref>PMID:15772804</ref> <ref>PMID:18837007</ref> <ref>PMID:21115670</ref> <ref>PMID:22508176</ref>
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[https://www.uniprot.org/uniprot/PKD2_HUMAN PKD2_HUMAN] Defects in PKD2 are the cause of polycystic kidney disease 2 (PKD2) [MIM:[https://omim.org/entry/613095 613095]. PKD2 is a disorder characterized by progressive formation and enlargement of cysts in both kidneys, typically leading to end-stage renal disease in adult life. Cysts also occurs in the liver and other organs. It represents approximately 15% of the cases of autosomal dominant polycystic kidney disease. PKD2 is clinically milder than PKD1 but it has a deleterious impact on overall life expectancy.<ref>PMID:9326320</ref> <ref>PMID:10541293</ref> <ref>PMID:10411676</ref> <ref>PMID:10835625</ref> <ref>PMID:11968093</ref> <ref>PMID:12707387</ref> <ref>PMID:14993477</ref> <ref>PMID:15772804</ref> <ref>PMID:21115670</ref>
== Function ==
== Function ==
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[[http://www.uniprot.org/uniprot/PKD2_HUMAN PKD2_HUMAN]] Involved in fluid-flow mechanosensation by the primary cilium in renal epithelium (By similarity). PKD1 and PKD2 may function through a common signaling pathway that is necessary for normal tubulogenesis (By similarity). Acts as a regulator of cilium length, together with PKD1 (By similarity). The dynamic control of cilium length is essential in the regulation of mechanotransductive signaling. The cilium length response creates a negative feedback loop whereby fluid shear-mediated deflection of the primary cilium, which decreases intracellular cAMP, leads to cilium shortening and thus decreases flow-induced signaling (By similarity). Functions as a calcium permeable cation channel. [[http://www.uniprot.org/uniprot/PKD1_HUMAN PKD1_HUMAN]] Involved in renal tubulogenesis. Involved in fluid-flow mechanosensation by the primary cilium in renal epithelium (By similarity). Acts as a regulator of cilium length, together with PKD2 (By similarity). The dynamic control of cilium length is essential in the regulation of mechanotransductive signaling. The cilium length response creates a negative feedback loop whereby fluid shear-mediated deflection of the primary cilium, which decreases intracellular cAMP, leads to cilium shortening and thus decreases flow-induced signaling (By similarity). May be an ion-channel regulator. Involved in adhesive protein-protein and protein-carbohydrate interactions.<ref>PMID:12482949</ref>
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[https://www.uniprot.org/uniprot/PKD2_HUMAN PKD2_HUMAN] Involved in fluid-flow mechanosensation by the primary cilium in renal epithelium (By similarity). PKD1 and PKD2 may function through a common signaling pathway that is necessary for normal tubulogenesis (By similarity). Acts as a regulator of cilium length, together with PKD1 (By similarity). The dynamic control of cilium length is essential in the regulation of mechanotransductive signaling. The cilium length response creates a negative feedback loop whereby fluid shear-mediated deflection of the primary cilium, which decreases intracellular cAMP, leads to cilium shortening and thus decreases flow-induced signaling (By similarity). Functions as a calcium permeable cation channel.
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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Mutations in two genes, pkd1 and pkd2, account for most cases of autosomal dominant polycystic kidney disease (ADPKD), one of the most common monogenetic disorders. Here we report the 3.6 A cryo-EM structure of truncated human PKD1/PKD2 complex assembled in a 1:3 ratio. PKD1 contains a voltage-gated ion channel (VGIC) fold that interacts with PKD2 to form the domain-swapped, yet non-canonical, TRP channel architecture. The S6 helix in PKD1 is broken in the middle, with the extracellular S6a resembling pore helix 1 in a typical TRP channel. Three positively charged cavity-facing residues on S6b may block cation permeation. In addition to VGIC, a 5-TM domain and a cytosolic PLAT domain were resolved in PKD1. The PKD1/PKD2 complex structure establishes a framework for dissecting the function and disease mechanisms of the PKD proteins.
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Structure of the human PKD1/PKD2 complex.,Su Q, Hu F, Ge X, Lei J, Yu S, Wang T, Zhou Q, Mei C, Shi Y Science. 2018 Aug 9. pii: science.aat9819. doi: 10.1126/science.aat9819. PMID:30093605<ref>PMID:30093605</ref>
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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</div>
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<div class="pdbe-citations 6a70" style="background-color:#fffaf0;"></div>
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== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
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</StructureSection>
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</SX>
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[[Category: Human]]
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[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Large Structures]]
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[[Category: Ge, X]]
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[[Category: Ge X]]
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[[Category: Hu, F]]
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[[Category: Hu F]]
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[[Category: Lei, J]]
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[[Category: Lei J]]
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[[Category: Mei, C]]
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[[Category: Mei C]]
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[[Category: Shi, Y]]
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[[Category: Shi Y]]
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[[Category: Su, Q]]
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[[Category: Su Q]]
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[[Category: Wang, T]]
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[[Category: Wang T]]
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[[Category: Yu, S]]
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[[Category: Yu S]]
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[[Category: Zhou, Q]]
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[[Category: Zhou Q]]
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[[Category: Asymmetric complex]]
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[[Category: Membrane protein]]
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[[Category: Polycystic kidney disease]]
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Current revision

Structure of the human PKD1/PKD2 complex

6a70, resolution 3.60Å

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