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http://purl.uniprot.org/citations/16400024http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/16400024http://www.w3.org/2000/01/rdf-schema#comment"Mouse models for autosomal-dominant polycystic kidney disease (ADPKD), derived from homozygous targeted disruption of Pkd1 gene, generally die in utero or perinatally because of systemic defects. We introduced a loxP site and a loxP-flanked mc1-neo cassette into introns 30 and 34, respectively, of the Pkd1 locus to generate a conditional, targeted mutation. Significantly, before excision of the floxed exons and mc1-neo from the targeted locus by Cre recombinase, mice homozygous for the targeted allele appeared normal at birth but developed polycystic kidney disease with a slower progression than that of Pkd-null mice. Further, the homozygotes continued to produce low levels of full-length Pkd1-encoded protein, suggesting that slight Pkd1 expression is sufficient for renal cyst formation in ADPKD. In this viable model, up-regulation of heparin-binding epidermal growth factor-like growth factor accompanied increased epidermal growth factor receptor signaling, which may be involved in abnormal proliferation of the cyst-lining epithelia. Increased apoptosis in cyst epithelia was only observed in the later period that correlated with the cyst regression. Abnormalities in Na(+)/K(+)-ATPase, aquaporin-2, and vasopressin V2 receptor expression were also identified. This mouse model may be suitable for further studies of progression and therapeutic interventions of ADPKD."xsd:string
http://purl.uniprot.org/citations/16400024http://purl.org/dc/terms/identifier"doi:10.2353/ajpath.2006.050342"xsd:string
http://purl.uniprot.org/citations/16400024http://purl.uniprot.org/core/author"Li H."xsd:string
http://purl.uniprot.org/citations/16400024http://purl.uniprot.org/core/author"Lin Y.T."xsd:string
http://purl.uniprot.org/citations/16400024http://purl.uniprot.org/core/author"Wang E."xsd:string
http://purl.uniprot.org/citations/16400024http://purl.uniprot.org/core/author"Jiang S.T."xsd:string
http://purl.uniprot.org/citations/16400024http://purl.uniprot.org/core/author"Lin H.K."xsd:string
http://purl.uniprot.org/citations/16400024http://purl.uniprot.org/core/author"Wang C.K."xsd:string
http://purl.uniprot.org/citations/16400024http://purl.uniprot.org/core/author"Chiou Y.Y."xsd:string
http://purl.uniprot.org/citations/16400024http://purl.uniprot.org/core/author"Tang M.J."xsd:string
http://purl.uniprot.org/citations/16400024http://purl.uniprot.org/core/author"Chi Y.C."xsd:string
http://purl.uniprot.org/citations/16400024http://purl.uniprot.org/core/date"2006"xsd:gYear
http://purl.uniprot.org/citations/16400024http://purl.uniprot.org/core/name"Am J Pathol"xsd:string
http://purl.uniprot.org/citations/16400024http://purl.uniprot.org/core/pages"205-220"xsd:string
http://purl.uniprot.org/citations/16400024http://purl.uniprot.org/core/title"Defining a link with autosomal-dominant polycystic kidney disease in mice with congenitally low expression of Pkd1."xsd:string
http://purl.uniprot.org/citations/16400024http://purl.uniprot.org/core/volume"168"xsd:string
http://purl.uniprot.org/citations/16400024http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/16400024
http://purl.uniprot.org/citations/16400024http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/16400024
http://purl.uniprot.org/uniprot/#_A0A0G2JDT8-mappedCitation-16400024http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/16400024
http://purl.uniprot.org/uniprot/#_A0A0A6YX05-mappedCitation-16400024http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/16400024
http://purl.uniprot.org/uniprot/#_D3Z2I8-mappedCitation-16400024http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/16400024
http://purl.uniprot.org/uniprot/#_D3Z5U6-mappedCitation-16400024http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/16400024
http://purl.uniprot.org/uniprot/#_D3YXZ9-mappedCitation-16400024http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/16400024
http://purl.uniprot.org/uniprot/#_P14094-mappedCitation-16400024http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/16400024