1b4r: Difference between revisions
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{{STRUCTURE_1b4r| PDB=1b4r | SCENE= }} | {{STRUCTURE_1b4r| PDB=1b4r | SCENE= }} | ||
===PKD DOMAIN 1 FROM HUMAN POLYCYSTEIN-1=== | |||
{{ABSTRACT_PUBMED_9889186}} | |||
=== | ==Disease== | ||
[[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> | |||
==Function== | |||
[[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> | |||
==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
<ref group="xtra">PMID:009889186</ref><references group="xtra"/> | <ref group="xtra">PMID:009889186</ref><references group="xtra"/><references/> | ||
[[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]] | ||
