THE HAN-SPRD RAT IS NOT A GENETIC MODEL OF HUMAN AUTOSOMAL-DOMINANT POLYCYSTIC KIDNEY-DISEASE TYPE-1

Human autosomal dominant polycystic kidney disease (ADPKD) is a high i ncidence disorder, leading to renal failure in many patients. The majo rity 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 genet ic 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 bac kcross of the offspring with BE yielded animals informative for linkag e analysis. This analysis revealed random segregation of the defect an d the Prm1 marker, indicating that the model is not caused by a mutati on in the PKD1 gene. We conclude that the Han:SPRD-Pkd rat strain is n ot a genetic model of PKD1.