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http://purl.uniprot.org/citations/21357692http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/21357692http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/21357692http://www.w3.org/2000/01/rdf-schema#comment"Nephronophthisis is the most common genetic cause of end-stage renal failure during childhood and adolescence. Genetic studies have identified disease-causing mutations in at least 11 different genes (NPHP1-11), but the function of the corresponding nephrocystin proteins remains poorly understood. The two evolutionarily conserved proteins nephrocystin-1 (NPHP1) and nephrocystin-4 (NPHP4) interact and localize to cilia in kidney, retina, and brain characterizing nephronophthisis and associated pathologies as result of a ciliopathy. Here we show that NPHP4, but not truncating patient mutations, negatively regulates tyrosine phosphorylation of NPHP1. NPHP4 counteracts Pyk2-mediated phosphorylation of three defined tyrosine residues of NPHP1 thereby controlling binding of NPHP1 to the trans-Golgi sorting protein PACS-1. Knockdown of NPHP4 resulted in an accumulation of NPHP1 in trans-Golgi vesicles of ciliated retinal epithelial cells. These data strongly suggest that NPHP4 acts upstream of NPHP1 in a common pathway and support the concept of a role for nephrocystin proteins in intracellular vesicular transport."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.org/dc/terms/identifier"doi:10.1074/jbc.m110.165464"xsd:string
http://purl.uniprot.org/citations/21357692http://purl.org/dc/terms/identifier"doi:10.1074/jbc.m110.165464"xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Benzing T."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Benzing T."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Schermer B."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Schermer B."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Walz G."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Walz G."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Dafinger C."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Dafinger C."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Zentgraf H."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Zentgraf H."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Liebau M.C."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Liebau M.C."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Blaukat A."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Blaukat A."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Habbig S."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Habbig S."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Bartram M.P."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Bartram M.P."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Burst V."xsd:string
http://purl.uniprot.org/citations/21357692http://purl.uniprot.org/core/author"Burst V."xsd:string