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rdf:type |
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lifeskim:mentions |
umls-concept:C0022646,
umls-concept:C0035820,
umls-concept:C0205145,
umls-concept:C0205224,
umls-concept:C0256073,
umls-concept:C0332208,
umls-concept:C0596311,
umls-concept:C0597304,
umls-concept:C0678594,
umls-concept:C0682972,
umls-concept:C1330957
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pubmed:issue |
47
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pubmed:dateCreated |
2007-11-27
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pubmed:abstractText |
Polycystin-1 (PC1) has an essential function in renal tubular morphogenesis and disruption of its function causes cystogenesis in human autosomal dominant polycystic kidney disease. We have previously shown that recombinant human PC1 is cis-autoproteolytically cleaved at the G protein-coupled receptor proteolytic site domain. To investigate the role of cleavage in vivo, we generated by gene targeting a Pkd1 knockin mouse (Pkd1(V/V)) that expresses noncleavable PC1. The Pkd1(V/V) mice show a hypomorphic phenotype, characterized by a delayed onset and distal nephron segment involvement of cystogenesis at postnatal maturation stage. We show that PC1 is ubiquitously and incompletely cleaved in wild-type mice, so that uncleaved and cleaved PC1 molecules coexist. Our study establishes a critical but restricted role of cleavage for PC1 function and suggests a differential function of the two types of PC1 molecules in vivo.
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pubmed:grant |
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-10469603,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-10490104,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-10677526,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-10861291,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-10968363,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-11245774,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-11593033,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-12482949,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-14718571,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-15008854,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-15545994,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-15568990,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-15579506,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-16261614,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-16399078,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-16932388,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-1711218,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-17502995,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-17525154,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-3794309,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-4209495,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-713285,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-7663510,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-8321262,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-8650242,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-8743483,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-8853424,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-8981910,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-9107672,
http://linkedlifedata.com/resource/pubmed/commentcorrection/18003909-9326937
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Nov
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pubmed:issn |
1091-6490
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pubmed:author |
pubmed-author:GaoJiangangJ,
pubmed-author:GaoJianggangJ,
pubmed-author:García GonzálezMiguel AMA,
pubmed-author:García-GonzálezMiguel AMA,
pubmed-author:GerminoGregory GGG,
pubmed-author:HackmannKarlK,
pubmed-author:HeXiaobingX,
pubmed-author:MenezesLuis FLF,
pubmed-author:PiontekKlausK,
pubmed-author:QianFengF,
pubmed-author:XuHangxueH,
pubmed-author:YuShengqiangS,
pubmed-author:ZuoJianJ
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pubmed:issnType |
Electronic
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pubmed:day |
20
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pubmed:volume |
104
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
18688-93
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pubmed:dateRevised |
2009-11-18
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pubmed:meshHeading |
pubmed-meshheading:18003909-Aging,
pubmed-meshheading:18003909-Animals,
pubmed-meshheading:18003909-Animals, Newborn,
pubmed-meshheading:18003909-Base Sequence,
pubmed-meshheading:18003909-Cells, Cultured,
pubmed-meshheading:18003909-Gene Deletion,
pubmed-meshheading:18003909-Gene Expression Regulation, Developmental,
pubmed-meshheading:18003909-Genotype,
pubmed-meshheading:18003909-Kidney Diseases, Cystic,
pubmed-meshheading:18003909-Kidney Tubules,
pubmed-meshheading:18003909-Mice,
pubmed-meshheading:18003909-Mice, Transgenic,
pubmed-meshheading:18003909-Receptors, G-Protein-Coupled,
pubmed-meshheading:18003909-TRPP Cation Channels
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pubmed:year |
2007
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pubmed:articleTitle |
Essential role of cleavage of Polycystin-1 at G protein-coupled receptor proteolytic site for kidney tubular structure.
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pubmed:affiliation |
Department of Medicine, Division of Nephrology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't,
Research Support, N.I.H., Extramural
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