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Pkdr1_mapped  -  polycystic kidney disease rat 1 (mapped)

Rattus norvegicus

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Disease relevance of Pkdr1_mapped


Psychiatry related information on Pkdr1_mapped


High impact information on Pkdr1_mapped


Chemical compound and disease context of Pkdr1_mapped


Biological context of Pkdr1_mapped


Anatomical context of Pkdr1_mapped

  • Alterations in basement membrane components, notably proteoglycans, in a rat model of polycystic kidney disease have been investigated [16].
  • Analyzing tissue sections from patients with typical autosomal dominant PKD demonstrated a high ET-1 expression within the epithelial cells of the cysts as well [17].
  • The presence of cathepsins could be demonstrated in cyst fluid from homozygous PKD rats and urinary excretion of cathepsins was enhanced in heterozygous animals [18].
  • The decrease in cathepsins in PKD was specific for tubules, as enzyme activities in glomeruli and liver tissue were unchanged and limited to the lysosomal compartment, since marker enzymes for cytoplasm, endoplasmatic reticulum and mitochondria were all normal [18].
  • However, it has long been known that the photoreceptor-bearing outer segments of retinal rods and cones are modified primary cilia and it has recently been found that kidney cells' primary cilia are sensitive flowmeters the disabling of which causes polycystic kidney disease [19].

Associations of Pkdr1_mapped with chemical compounds

  • This article reports the positional cloning and mutation analysis of the rat PKD gene, which revealed a C to T transition that replaces an arginine by a tryptophan at amino acid 823 in the protein sequence [1].
  • Basement membrane chondroitin sulfate proteoglycan alterations in a rat model of polycystic kidney disease [16].
  • This study determined whether ingestion of potassium citrate and citric acid would ameliorate PKD [20].
  • Healthy normal and heterozygous littermate Han:SPRD rats with autosomal dominant PKD were provided with either tap water or 55 mM K3citrate/67 mM citric acid solution (KCitr) to drink starting at the age of 1 mo [20].
  • The administration of ammonium chloride or of sodium or potassium bicarbonate has marked effects on the development of polycystic kidney disease (PKD) in Han:SPRD rats [21].

Regulatory relationships of Pkdr1_mapped


Other interactions of Pkdr1_mapped


Analytical, diagnostic and therapeutic context of Pkdr1_mapped


  1. Missense mutation in sterile alpha motif of novel protein SamCystin is associated with polycystic kidney disease in (cy/+) rat. Brown, J.H., Bihoreau, M.T., Hoffmann, S., Kränzlin, B., Tychinskaya, I., Obermüller, N., Podlich, D., Boehn, S.N., Kaisaki, P.J., Megel, N., Danoy, P., Copley, R.R., Broxholme, J., Witzgall, R., Lathrop, M., Gretz, N., Gauguier, D. J. Am. Soc. Nephrol. (2005) [Pubmed]
  2. Caspase inhibition reduces tubular apoptosis and proliferation and slows disease progression in polycystic kidney disease. Tao, Y., Kim, J., Faubel, S., Wu, J.C., Falk, S.A., Schrier, R.W., Edelstein, C.L. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  3. Characterization of a major modifier locus for polycystic kidney disease (Modpkdr1) in the Han:SPRD(cy/+) rat in a region conserved with a mouse modifier locus for Alport syndrome. Bihoreau, M.T., Megel, N., Brown, J.H., Kränzlin, B., Crombez, L., Tychinskaya, Y., Broxholme, J., Kratz, S., Bergmann, V., Hoffman, S., Gauguier, D., Gretz, N. Hum. Mol. Genet. (2002) [Pubmed]
  4. Polycystic kidney rat is a novel animal model of Caroli's disease associated with congenital hepatic fibrosis. Sanzen, T., Harada, K., Yasoshima, M., Kawamura, Y., Ishibashi, M., Nakanuma, Y. Am. J. Pathol. (2001) [Pubmed]
  5. Hereditary polycystic kidney disease. Adult polycystic kidney disease associated with renal hypertension, renal osteodystrophy, and uremic enteritis in SPRD rats. Kaspareit-Rittinghausen, J., Deerberg, F., Wcislo, A. Am. J. Pathol. (1991) [Pubmed]
  6. Dietary conjugated linoleic acid reduces PGE2 release and interstitial injury in rat polycystic kidney disease. Ogborn, M.R., Nitschmann, E., Bankovic-Calic, N., Weiler, H.A., Fitzpatrick-Wong, S., Aukema, H.M. Kidney Int. (2003) [Pubmed]
  7. Defective planar cell polarity in polycystic kidney disease. Fischer, E., Legue, E., Doyen, A., Nato, F., Nicolas, J.F., Torres, V., Yaniv, M., Pontoglio, M. Nat. Genet. (2006) [Pubmed]
  8. Tuberin-dependent membrane localization of polycystin-1: a functional link between polycystic kidney disease and the TSC2 tumor suppressor gene. Kleymenova, E., Ibraghimov-Beskrovnaya, O., Kugoh, H., Everitt, J., Xu, H., Kiguchi, K., Landes, G., Harris, P., Walker, C. Mol. Cell (2001) [Pubmed]
  9. Defects in cholangiocyte fibrocystin expression and ciliary structure in the PCK rat. Masyuk, T.V., Huang, B.Q., Ward, C.J., Masyuk, A.I., Yuan, D., Splinter, P.L., Punyashthiti, R., Ritman, E.L., Torres, V.E., Harris, P.C., LaRusso, N.F. Gastroenterology (2003) [Pubmed]
  10. Effectiveness of vasopressin V2 receptor antagonists OPC-31260 and OPC-41061 on polycystic kidney disease development in the PCK rat. Wang, X., Gattone, V., Harris, P.C., Torres, V.E. J. Am. Soc. Nephrol. (2005) [Pubmed]
  11. EGF receptor tyrosine kinase inhibition attenuates the development of PKD in Han:SPRD rats. Torres, V.E., Sweeney, W.E., Wang, X., Qian, Q., Harris, P.C., Frost, P., Avner, E.D. Kidney Int. (2003) [Pubmed]
  12. Hypertension and renal injury in experimental polycystic kidney disease. Kennefick, T.M., Al-Nimri, M.A., Oyama, T.T., Thompson, M.M., Kelly, F.J., Chapman, J.G., Anderson, S. Kidney Int. (1999) [Pubmed]
  13. Methylprednisolone retards the progression of inherited polycystic kidney disease in rodents. Gattone, V.H., Cowley, B.D., Barash, B.D., Nagao, S., Takahashi, H., Yamaguchi, T., Grantham, J.J. Am. J. Kidney Dis. (1995) [Pubmed]
  14. Dietary betaine modifies hepatic metabolism but not renal injury in rat polycystic kidney disease. Ogborn, M.R., Nitschmann, E., Bankovic-Calic, N., Buist, R., Peeling, J. Am. J. Physiol. Gastrointest. Liver Physiol. (2000) [Pubmed]
  15. Downregulation of Ke 6, a novel gene encoded within the major histocompatibility complex, in murine polycystic kidney disease. Aziz, N., Maxwell, M.M., St Jacques, B., Brenner, B.M. Mol. Cell. Biol. (1993) [Pubmed]
  16. Basement membrane chondroitin sulfate proteoglycan alterations in a rat model of polycystic kidney disease. Ehara, T., Carone, F.A., McCarthy, K.J., Couchman, J.R. Am. J. Pathol. (1994) [Pubmed]
  17. Renal endothelin system in polycystic kidney disease. Hocher, B., Zart, R., Schwarz, A., Vogt, V., Braun, C., Thöne-Reineke, C., Braun, N., Neumayer, H.H., Koppenhagen, K., Bauer, C., Rohmeiss, P. J. Am. Soc. Nephrol. (1998) [Pubmed]
  18. Alterations of cathepsins B, H and L in proximal tubules from polycystic kidneys of the Han:SPRD rat. Schaefer, L., Han, X., Gretz, N., Schaefer, R.M. Kidney Int. (1996) [Pubmed]
  19. The neuronal primary cilium--an extrasynaptic signaling device. Whitfield, J.F. Cell. Signal. (2004) [Pubmed]
  20. Potassium citrate/citric acid intake improves renal function in rats with polycystic kidney disease. Tanner, G.A. J. Am. Soc. Nephrol. (1998) [Pubmed]
  21. Aggravation of polycystic kidney disease in Han:SPRD rats by buthionine sulfoximine. Torres, V.E., Bengal, R.J., Litwiller, R.D., Wilson, D.M. J. Am. Soc. Nephrol. (1997) [Pubmed]
  22. Genesis of renal cysts is associated with clusterin expression in experimental cystic disease. Rosenberg, M.E., Manivel, J.C., Carone, F.A., Kanwar, Y.S. J. Am. Soc. Nephrol. (1995) [Pubmed]
  23. Tubular gelatinase A (MMP-2) and its tissue inhibitors in polycystic kidney disease in the Han:SPRD rat. Schaefer, L., Han, X., Gretz, N., Häfner, C., Meier, K., Matzkies, F., Schaefer, R.M. Kidney Int. (1996) [Pubmed]
  24. Polycystin-2 expression is increased following experimental ischaemic renal injury. Zhao, Y., Haylor, J.L., Ong, A.C. Nephrol. Dial. Transplant. (2002) [Pubmed]
  25. Androgen receptor pathway in rats with autosomal dominant polycystic kidney disease. Nagao, S., Kusaka, M., Nishii, K., Marunouchi, T., Kurahashi, H., Takahashi, H., Grantham, J. J. Am. Soc. Nephrol. (2005) [Pubmed]
  26. Activity and functional significance of the renal kallikrein-kinin-system in polycystic kidney disease of the rat. Braun, C., Kleemann, T., Hilgenfeldt, U., Riester, U., Rohmeiss, P., van der Woude, F.J. Kidney Int. (2002) [Pubmed]
  27. Retention of membrane-localized beta-catenin in cells lacking functional polycystin-1 and tuberin. Kugoh, H., Kleymenova, E., Walker, C.L. Mol. Carcinog. (2002) [Pubmed]
  28. Accelerated renal death after unilateral nephrectomy in a rat strain suffering from autosomal dominant polycystic kidney disease. Gretz, N. J. Am. Soc. Nephrol. (1994) [Pubmed]
  29. Gene delivery in renal tubular epithelial cells using recombinant adeno-associated viral vectors. Chen, S., Agarwal, A., Glushakova, O.Y., Jorgensen, M.S., Salgar, S.K., Poirier, A., Flotte, T.R., Croker, B.P., Madsen, K.M., Atkinson, M.A., Hauswirth, W.W., Berns, K.I., Tisher, C.C. J. Am. Soc. Nephrol. (2003) [Pubmed]
  30. Gender-dependent disease severity in autosomal polycystic kidney disease of rats. Gretz, N., Ceccherini, I., Kränzlin, B., Klöting, I., Devoto, M., Rohmeiss, P., Hocher, B., Waldherr, R., Romeo, G. Kidney Int. (1995) [Pubmed]
  31. Tubular basement membrane changes during induction and regression of drug-induced polycystic kidney disease. Carone, F.A., Butkowski, R.J., Nakamura, S., Polenakovic, M., Kanwar, Y.S. Kidney Int. (1994) [Pubmed]
  32. Characterization of the Han:SPRD rat model for hereditary polycystic kidney disease. Schäfer, K., Gretz, N., Bader, M., Oberbäumer, I., Eckardt, K.U., Kriz, W., Bachmann, S. Kidney Int. (1994) [Pubmed]
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