title: Rat models of autosomal dominant polycystic kidney disease.
abstract: Several rat models of polycystic kidney disease (PKD) have been published. The only rat model of autosomal dominant polycystic kidney disease currently used is the so-called Hannover rat (Han:SPRD cy/+). This model is characterized by a slow progression of uraemia, proteinuria and hyperlipidaemia. Histological changes clearly resemble those seen is human PKD. The localization of Na+/K(+)-ATPase correlating with the phenotype of the cysts--basal in moderately expanded and apical in highly expanded cysts--suggests that the mislocation of the Na+/K(+)-ATPase is involved in the mechanism of cyst expansion rather than formation, and a consequence of cell dedifferentiation rather than an initial event. Of note is a considerable gender difference in disease severity. Disease anticipation or genetic imprinting does not occur. In addition to gender, a number of interventions influence the progression rate: acceleration is noted after unilateral nephrectomy, the induction of acidosis, chloride feeding or an increased protein intake; slowing down of the course occurs after the induction of alkalosis and castration, and after treatment with lovastatin and methylprednisolone. Thus the Han:SPRD cy/+ rat represents the only well-documented rat model of autosomal dominant PKD resembling a number of features of the human disease.
authors.affiliation: V. Medical Department, Klinikum Mannheim, University of Heidelberg, Germany.
authors.forename: N
authors.initials: N
authors.lastname: Gretz
authors.forename: B
authors.initials: B
authors.lastname: Kränzlin
authors.forename: R
authors.initials: R
authors.lastname: Pey
authors.forename: G
authors.initials: G
authors.lastname: Schieren
authors.forename: J
authors.initials: J
authors.lastname: Bach
authors.forename: N
authors.initials: N
authors.lastname: Obermüller
authors.forename: I
authors.initials: I
authors.lastname: Ceccherini
authors.forename: I
authors.initials: I
authors.lastname: Klöting
authors.forename: P
authors.initials: P
authors.lastname: Rohmeiss
authors.forename: S
authors.initials: S
authors.lastname: Bachmann
authors.forename: M
authors.initials: M
authors.lastname: Hafner
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D016891:Polycystic Kidney, Autosomal Dominant / Q000378:metabolism / Q000473:pathology / Q000662:veterinary*; D051381:Rats; D012376:Rodent Diseases / Q000378:metabolism* / Q000473:pathology*