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1b4r

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==PKD DOMAIN 1 FROM HUMAN POLYCYSTEIN-1==
==PKD DOMAIN 1 FROM HUMAN POLYCYSTEIN-1==
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<StructureSection load='1b4r' size='340' side='right'caption='[[1b4r]], [[NMR_Ensembles_of_Models | 20 NMR models]]' scene=''>
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<StructureSection load='1b4r' size='340' side='right'caption='[[1b4r]]' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[1b4r]] is a 1 chain structure. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1B4R OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1B4R FirstGlance]. <br>
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<table><tr><td colspan='2'>[[1b4r]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1B4R OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1B4R FirstGlance]. <br>
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</td></tr><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=1b4r FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1b4r OCA], [http://pdbe.org/1b4r PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=1b4r RCSB], [http://www.ebi.ac.uk/pdbsum/1b4r PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=1b4r ProSAT]</span></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">Solution NMR</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=1b4r FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1b4r OCA], [https://pdbe.org/1b4r PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1b4r RCSB], [https://www.ebi.ac.uk/pdbsum/1b4r PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1b4r ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
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[[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/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 ==
== Function ==
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[[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/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>
== Evolutionary Conservation ==
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
[[Image:Consurf_key_small.gif|200px|right]]
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__TOC__
__TOC__
</StructureSection>
</StructureSection>
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[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Large Structures]]
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[[Category: Bycroft, M]]
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[[Category: Bycroft M]]
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[[Category: Membrane protein]]
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[[Category: Pkd domain 1 from human polycystein-1]]
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Current revision

PKD DOMAIN 1 FROM HUMAN POLYCYSTEIN-1

PDB ID 1b4r

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