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http://purl.uniprot.org/citations/24158982http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/24158982http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/24158982http://www.w3.org/2000/01/rdf-schema#comment"cAMP is an important mediator of cystogenesis in polycystic kidney disease (PKD). Several adenylyl cyclase (AC) isoforms could mediate cAMP accumulation in PKD, and identification of a specific pathogenic AC isoform is of therapeutic interest. We investigated the role of AC6 in a mouse model of PKD that is homozygous for the loxP-flanked PKD1 gene and heterozygous for an aquaporin-2-Cre recombinase transgene to achieve collecting duct-specific gene targeting. Collecting duct-specific knockout of polycystin-1 caused massive renal cyst formation, kidney enlargement, and severe kidney failure, with a mean survival time of 2 months. In contrast, coincident collecting duct-specific knockout of polycystin-1 and AC6 (also homozygous for the floxed ADCY6 gene) markedly decreased kidney size and cystogenesis, improved renal function, reduced activation of the B-Raf/ERK/MEK pathway, and greatly increased survival. Absence of collecting duct AC6 did not alter urinary cAMP excretion or kidney cAMP concentration. In conclusion, AC6 is a key mediator of cyst formation and renal injury in a model of PKD."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.org/dc/terms/identifier"doi:10.1681/asn.2013010077"xsd:string
http://purl.uniprot.org/citations/24158982http://purl.org/dc/terms/identifier"doi:10.1681/asn.2013010077"xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/author"Rees S."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/author"Rees S."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/author"Kittikulsuth W."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/author"Kittikulsuth W."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/author"Kohan D.E."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/author"Kohan D.E."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/author"Roos K."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/author"Roos K."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/author"Strait K.A."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/author"Strait K.A."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/author"Van Hoek A."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/author"Van Hoek A."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/date"2014"xsd:gYear
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/date"2014"xsd:gYear
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/name"J. Am. Soc. Nephrol."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/name"J. Am. Soc. Nephrol."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/pages"232-237"xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/pages"232-237"xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/title"Adenylyl cyclase 6 deficiency ameliorates polycystic kidney disease."xsd:string
http://purl.uniprot.org/citations/24158982http://purl.uniprot.org/core/title"Adenylyl cyclase 6 deficiency ameliorates polycystic kidney disease."xsd:string