View source for 8zkt


==Structure of Polycystin-1/Polycystin-2 complex with GOF mutations==
<StructureSection load='8zkt' size='340' side='right'caption='[[8zkt]], [[Resolution|resolution]] 3.34&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[8zkt]] 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=8ZKT OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=8ZKT FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 3.34&#8491;</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene></td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=8zkt FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=8zkt OCA], [https://pdbe.org/8zkt PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=8zkt RCSB], [https://www.ebi.ac.uk/pdbsum/8zkt PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=8zkt ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/PKD1_HUMAN PKD1_HUMAN] Defects in PKD1 are the cause of polycystic kidney disease 1 (PKD1) [MIM:[https://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> 
== Function ==
[https://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> 
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Chen MY]]
[[Category: Shi YG]]
[[Category: Su Q]]