RGD Reference Report - Characterization of the Han:SPRD rat model for hereditary polycystic kidney disease. - Rat Genome Database

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Characterization of the Han:SPRD rat model for hereditary polycystic kidney disease.

Authors: Schafer, K  Gretz, N  Bader, M  Oberbaumer, I  Eckardt, KU  Kriz, W  Bachmann, S 
Citation: Schafer K, etal., Kidney Int 1994 Jul;46(1):134-52.
RGD ID: 1300446
Pubmed: PMID:7933831   (View Abstract at PubMed)

The Han:SPRD rat model for inherited polycystic kidney disease (PKD) was characterized (clinical parameters, morphology, immunohistochemistry and in situ hybridization). Homozygous animals died of uremia after three to four weeks with severe cystic transformation of virtually all nephrons and collecting ducts (serum urea: 616 +/- 195 mg/dl; kidney-to-body weight ratio: > 20%). In heterozygotes, slow progression of the disease led to death between the 12th and 21st month (median: 17 months; serum urea levels above 200 mg/dl). Kidney enlargement was moderate, and cysts were restricted to the cortex and outer medulla. Immunohistochemical markers showed that approximately 75% of the cysts were derived from the proximal tubule. Cystic transformation started in the proximal tubule with a sharp onset of basement membrane alteration and a loss of epithelial differentiation restricted to small focal areas. In these areas, alpha 1(IV) collagen and laminin B1 mRNA were enhanced as revealed by isotopic and non-isotopic in situ hybridization. Fibroblasts underlying the affected tubular portions were involved in matrix overexpression resulting in subepithelial accumulation of immunoreactive collagen IV and laminin. In later stages of cystic transformation distal nephron segments were affected as well. A reversal in epithelial polarity as judged from Na,K-ATPase-immunoreactivity was not observed. Renal immunoreactive renin-status was significantly decreased. Hematocrit was lowered in heterozygotes (40.4 +/- 5.8 vol% compared to 46.7 +/- 1.99 vol% in controls; P < 0.05) and total renal EPO mRNA was reduced to 36 +/- 14% of the mean value of control animals, whereas serum EPO levels were not significantly altered. We conclude that the Han:SPRD rat is a useful model for the study of human ADPKD since both diseases are similar in several aspects. The model is particularly suitable for the study of epithelial-mesenchymal interactions at the beginning of tubular cystic transformation.

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Phenotype Annotations    Click to see Annotation Detail View

Mammalian Phenotype

TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
decreased hematocrit  IAGP 1300446; 1300446; 1300446compared to PKDRGD 
enlarged kidney  IAGP 1300446; 1300446; 1300446 RGD 
increased blood urea nitrogen level  IAGP 1300446; 1300446; 1300446compared to PKDRGD 
increased circulating creatinine level  IAGP 1300446; 1300446; 1300446compared to PKDRGD 
increased kidney weight  IAGP 1300446; 1300446; 1300446compared to PKDRGD 
premature death  IAGP 1300446; 1300446; 1300446 RGD 
Objects Annotated

Genes (Rattus norvegicus)
Anks6  (ankyrin repeat and sterile alpha motif domain containing 6)
Anks6PKD  (ankyrin repeat and sterile alpha motif domain containing 6, polycystic kidney disease)

Genes (Mus musculus)
Anks6  (ankyrin repeat and sterile alpha motif domain containing 6)

Genes (Homo sapiens)
ANKS6  (ankyrin repeat and sterile alpha motif domain containing 6)

Strains
SPRD-Anks6PKD/Rrrc  (NA)

Objects referenced in this article
Strain PKD PKD Rattus norvegicus

Additional Information