1b4r

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[[Image:1b4r.jpg|left|200px]]
 
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==PKD DOMAIN 1 FROM HUMAN POLYCYSTEIN-1==
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The line below this paragraph, containing "STRUCTURE_1b4r", creates the "Structure Box" on the page.
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<StructureSection load='1b4r' size='340' side='right'caption='[[1b4r]]' scene=''>
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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== Structural highlights ==
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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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='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>
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{{STRUCTURE_1b4r| PDB=1b4r | SCENE= }}
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</table>
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== Disease ==
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'''PKD DOMAIN 1 FROM HUMAN POLYCYSTEIN-1'''
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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>
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== Function ==
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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>
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==Overview==
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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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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/b4/1b4r_consurf.spt"</scriptWhenChecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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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/main_output.php?pdb_ID=1b4r ConSurf].
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== Publication Abstract from PubMed ==
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.
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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==About this Structure==
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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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1B4R is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1B4R OCA].
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==Reference==
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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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:[http://www.ncbi.nlm.nih.gov/pubmed/9889186 9889186]
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</div>
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<div class="pdbe-citations 1b4r" style="background-color:#fffaf0;"></div>
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[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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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May 2 11:04:14 2008''
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Current revision

PKD DOMAIN 1 FROM HUMAN POLYCYSTEIN-1

PDB ID 1b4r

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