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.



RGD Manual Disease Annotations    Click to see Annotation Detail View

  
Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
ANKS6Humanpolycystic kidney disease  ISORGD:3334 RGD 
Anks6Ratpolycystic kidney disease  IAGP  RGD 
Anks6Mousepolycystic kidney disease  ISORGD:3334 RGD 
Anks6PKDRatpolycystic kidney disease  IAGP  RGD 
SPRD-Anks6PKD/RrrcRatpolycystic kidney disease  IAGP  RGD 
ANKS6Humanproteinuria  ISORGD:3334 RGD 
Anks6Mouseproteinuria  ISORGD:3334 RGD 
Anks6Ratproteinuria  IAGP  RGD 
Anks6PKDRatproteinuria  IAGP  RGD 
SPRD-Anks6PKD/RrrcRatproteinuria  IAGP  RGD 
ANKS6Humanuremia  ISORGD:3334 RGD 
Anks6Mouseuremia  ISORGD:3334 RGD 
Anks6Raturemia  IAGP  RGD 
Anks6PKDRaturemia  IAGP  RGD 
SPRD-Anks6PKD/RrrcRaturemia  IAGP  RGD 

Phenotype Annotations    Click to see Annotation Detail View

Mammalian Phenotype

Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
Anks6Ratdecreased hematocrit  IAGP compared to PKDRGD 
Anks6PKDRatdecreased hematocrit  IAGP compared to PKDRGD 
SPRD-Anks6PKD/RrrcRatdecreased hematocrit  IAGP compared to PKDRGD 
Anks6Ratenlarged kidney  IAGP  RGD 
Anks6PKDRatenlarged kidney  IAGP  RGD 
SPRD-Anks6PKD/RrrcRatenlarged kidney  IAGP  RGD 
Anks6Ratincreased blood urea nitrogen level  IAGP compared to PKDRGD 
Anks6PKDRatincreased blood urea nitrogen level  IAGP compared to PKDRGD 
SPRD-Anks6PKD/RrrcRatincreased blood urea nitrogen level  IAGP compared to PKDRGD 
Anks6Ratincreased circulating creatinine level  IAGP compared to PKDRGD 
Anks6PKDRatincreased circulating creatinine level  IAGP compared to PKDRGD 
SPRD-Anks6PKD/RrrcRatincreased circulating creatinine level  IAGP compared to PKDRGD 
Anks6Ratincreased kidney weight  IAGP compared to PKDRGD 
Anks6PKDRatincreased kidney weight  IAGP compared to PKDRGD 
SPRD-Anks6PKD/RrrcRatincreased kidney weight  IAGP compared to PKDRGD 
Anks6Ratpremature death  IAGP  RGD 
Anks6PKDRatpremature death  IAGP  RGD 
SPRD-Anks6PKD/RrrcRatpremature death  IAGP  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