title: The Han:SPRD rat is not a genetic model of human autosomal dominant polycystic kidney disease type 1.
abstract: Human autosomal dominant polycystic kidney disease (ADPKD) is a high incidence disorder leading to renal failure in many patients. The majority of cases results from a mutation in the PKD1 gene. The only well documented animal model of ADPKD is the Han:SPRD-Pkd strain. Its genetic basis is unknown as yet. In the current study we determined whether the disease in these rats is genetically linked to the rat homologue of the PKD1 gene. We used the protamine gene as a polymorphic marker (Prm1) of the PKD1 region. Matings of Han:SPRD-Pkd with BB rats and backcross of the offspring with BB yielded animals informative for linkage analysis. This analysis revealed random segregation of the defect and the Prm1 marker, indicating that the model is not caused by a mutation in the PKD1 gene. We conclude that the Han:SPRD-Pkd rat strain is not a genetic model of PKD1.
authors.affiliation: Department of Pediatrics, Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: J
authors.initials: J
authors.lastname: Nauta
authors.forename: M A
authors.initials: MA
authors.lastname: Goedbloed
authors.forename: T M
authors.initials: TM
authors.lastname: Luider
authors.forename: A T
authors.initials: AT
authors.lastname: Hoogeveen
authors.forename: A M
authors.initials: AM
authors.lastname: van den Ouweland
authors.forename: D J
authors.initials: DJ
authors.lastname: Halley
mesh_terms: D000818:Animals; D015139:Blotting, Southern; D004195:Disease Models, Animal; D005260:Female; D008040:Genetic Linkage / Q000235:genetics; D005819:Genetic Markers / Q000235:genetics; D005838:Genotype; D006579:Heterozygote; D006801:Humans; D007668:Kidney / Q000473:pathology*; D008126:Lod Score; D008297:Male; D010641:Phenotype; D016891:Polycystic Kidney, Autosomal Dominant / Q000235:genetics*; D011506:Proteins / Q000235:genetics*; D051381:Rats; D011922:Rats, Mutant Strains; D050396:TRPP Cation Channels
chemical_list: D005819:Genetic Markers; D011506:Proteins; D050396:TRPP Cation Channels