1b4r

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{{STRUCTURE_1b4r| PDB=1b4r | SCENE= }}
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==PKD DOMAIN 1 FROM HUMAN POLYCYSTEIN-1==
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===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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{{ABSTRACT_PUBMED_9889186}}
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== Structural highlights ==
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<table><tr><td colspan='2'>[[1b4r]] is a 1 chain structure with sequence from [http://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 [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1B4R FirstGlance]. <br>
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</td></tr><tr><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://www.rcsb.org/pdb/explore.do?structureId=1b4r RCSB], [http://www.ebi.ac.uk/pdbsum/1b4r PDBsum]</span></td></tr>
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<table>
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== 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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== 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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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/b4/1b4r_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/chain_selection.php?pdb_ID=2ata ConSurf].
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<div style="clear:both"></div>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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Most cases of autosomal dominant polycystic kidney disease (ADPKD) are the result of mutations in the PKD1 gene. The PKD1 gene codes for a large cell-surface glycoprotein, polycystin-1, of unknown function, which, based on its predicted domain structure, may be involved in protein-protein and protein-carbohydrate interactions. Approximately 30% of polycystin-1 consists of 16 copies of a novel protein module called the PKD domain. Here we show that this domain has a beta-sandwich fold. Although this fold is common to a number of cell-surface modules, the PKD domain represents a distinct protein family. The tenth PKD domain of human and Fugu polycystin-1 show extensive conservation of surface residues suggesting that this region could be a ligand-binding site. This structure will allow the likely effects of missense mutations in a large part of the PKD1 gene to be determined.
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==Disease==
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The structure of a PKD domain from polycystin-1: implications for polycystic kidney disease.,Bycroft M, Bateman A, Clarke J, Hamill SJ, Sandford R, Thomas RL, Chothia C EMBO J. 1999 Jan 15;18(2):297-305. PMID:9889186<ref>PMID:9889186</ref>
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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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==Function==
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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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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</div>
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== References ==
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==About this Structure==
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<references/>
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[[1b4r]] is a 1 chain structure with sequence from [http://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].
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__TOC__
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</StructureSection>
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==Reference==
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<ref group="xtra">PMID:009889186</ref><references group="xtra"/><references/>
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Bycroft, M.]]
[[Category: Bycroft, M.]]
[[Category: Membrane protein]]
[[Category: Membrane protein]]
[[Category: Pkd domain 1 from human polycystein-1]]
[[Category: Pkd domain 1 from human polycystein-1]]

Revision as of 16:42, 29 September 2014

PKD DOMAIN 1 FROM HUMAN POLYCYSTEIN-1

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