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MeSH Review

Polycystic Kidney, Autosomal Dominant

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Disease relevance of Polycystic Kidney, Autosomal Dominant


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High impact information on Polycystic Kidney, Autosomal Dominant

  • Somatic loss of Pkd2 expression is both necessary and sufficient for renal cyst formation in ADPKD, suggesting that PKD2 occurs by a cellular recessive mechanism [7].
  • Here we show, in a mouse model of ADPKD (Pkd2(-/tm1Som)), a similar cellular phenotype and response to OPC31260 treatment, with reduction of renal cyclic AMP (cAMP) levels, prevention of renal enlargement, marked inhibition of cystogenesis and protection of renal function [8].
  • Polycystin-2, the product of the second gene mutated in ADPKD, modulates the signaling properties of the polycystin-1 CTT [9].
  • The search for the adult polycystic kidney disease gene has recently been narrowed to the analysis of candidate loci on chromosome 16, and localization of the gene determining juvenile Batten disease has been further refined by disequilibrium mapping [10].
  • Non-PKD1 ADPKD has a much milder phenotype than that linked to PKD1 [11].

Chemical compound and disease context of Polycystic Kidney, Autosomal Dominant

  • In the Han:SPRD (cy/+) model for ADPKD, the proximal nephron is primarily affected by the cystic changes [12].
  • Treatment of ADPKD cells or ARPKD cells with either Bay K8644, a Ca2+ channel activator, or A23187, a Ca2+ ionophore, caused sustained increases in intracellular Ca2+ levels and completely reversed the mitogenic response to cAMP [13].
  • (35S) sulfate incorporation studies revealed that, among various GF, HGF and TGF-beta 1 had the maximal stimulatory effect on the synthesis of PG extracted from ADPKD cells [14].
  • Effects of angiotensin converting enzyme inhibition in adult polycystic kidney disease [15].
  • The mean SI was not significantly different in patients with renal disease of immune (IgAGN) or non-immune (ADPKD) origin, and it was not correlated with GFR (r = 0.01, P < 0.52), intact PTH (r = -0.23, P < 0.11) or calcitriol concentration (r = -0.03, P < 0.82) [16].

Biological context of Polycystic Kidney, Autosomal Dominant


Anatomical context of Polycystic Kidney, Autosomal Dominant


Gene context of Polycystic Kidney, Autosomal Dominant


Analytical, diagnostic and therapeutic context of Polycystic Kidney, Autosomal Dominant


  1. An autosomal recessive polycystic kidney disease gene homolog is involved in intraflagellar transport in C. elegans ciliated sensory neurons. Qin, H., Rosenbaum, J.L., Barr, M.M. Curr. Biol. (2001) [Pubmed]
  2. An unbalanced submicroscopic translocation t(8;16)(q24.3;p13.3)pat associated with tuberous sclerosis complex, adult polycystic kidney disease, and hypomelanosis of Ito. Eussen, B.H., Bartalini, G., Bakker, L., Balestri, P., Di Lucca, C., Van Hemel, J.O., Dauwerse, H., van Den Ouweland, A.M., Ris-Stalpers, C., Verhoef, S., Halley, D.J., Fois, A. J. Med. Genet. (2000) [Pubmed]
  3. Dynamics of erythropoiesis following renal transplantation. Besarab, A., Caro, J., Jarrell, B.E., Francos, G., Erslev, A.J. Kidney Int. (1987) [Pubmed]
  4. CD2-associated protein directly interacts with the actin cytoskeleton. Lehtonen, S., Zhao, F., Lehtonen, E. Am. J. Physiol. Renal Physiol. (2002) [Pubmed]
  5. Observations in a Saudi-Arabian dialysis population over a 13-year period. Hussein, M.M., Mooij, J.M., Roujouleh, H., el-Sayed, H. Nephrol. Dial. Transplant. (1994) [Pubmed]
  6. DNA probe technology: implications for service planning in Britain. Rona, R.J., Swan, A.V., Beech, R., Wilson, O.M., Kavanagh, F.B., Brown, C., Axtell, C., Mandalia, S. Clin. Genet. (1992) [Pubmed]
  7. Somatic inactivation of Pkd2 results in polycystic kidney disease. Wu, G., D'Agati, V., Cai, Y., Markowitz, G., Park, J.H., Reynolds, D.M., Maeda, Y., Le, T.C., Hou, H., Kucherlapati, R., Edelmann, W., Somlo, S. Cell (1998) [Pubmed]
  8. Effective treatment of an orthologous model of autosomal dominant polycystic kidney disease. Torres, V.E., Wang, X., Qian, Q., Somlo, S., Harris, P.C., Gattone, V.H. Nat. Med. (2004) [Pubmed]
  9. Mechanical stimuli induce cleavage and nuclear translocation of the polycystin-1 C terminus. Chauvet, V., Tian, X., Husson, H., Grimm, D.H., Wang, T., Hiesberger, T., Hieseberger, T., Igarashi, P., Bennett, A.M., Ibraghimov-Beskrovnaya, O., Somlo, S., Caplan, M.J. J. Clin. Invest. (2004) [Pubmed]
  10. Integrating maps of chromosome 16. Mulley, J.C., Sutherland, G.R. Curr. Opin. Genet. Dev. (1993) [Pubmed]
  11. Phenotype and genotype heterogeneity in autosomal dominant polycystic kidney disease. Ravine, D., Walker, R.G., Gibson, R.N., Forrest, S.M., Richards, R.I., Friend, K., Sheffield, L.J., Kincaid-Smith, P., Danks, D.M. Lancet (1992) [Pubmed]
  12. Altered expression of type II sodium/phosphate cotransporter in polycystic kidney disease. Vogel, M., Kränzlin, B., Biber, J., Murer, H., Gretz, N., Bachmann, S. J. Am. Soc. Nephrol. (2000) [Pubmed]
  13. Calcium restores a normal proliferation phenotype in human polycystic kidney disease epithelial cells. Yamaguchi, T., Hempson, S.J., Reif, G.A., Hedge, A.M., Wallace, D.P. J. Am. Soc. Nephrol. (2006) [Pubmed]
  14. Differential growth factor-induced modulation of proteoglycans synthesized by normal human renal versus cyst-derived cells. Kovacs, J., Carone, F.A., Liu, Z.Z., Nakumara, S., Kumar, A., Kanwar, Y.S. J. Am. Soc. Nephrol. (1994) [Pubmed]
  15. Effects of angiotensin converting enzyme inhibition in adult polycystic kidney disease. Watson, M.L., Macnicol, A.M., Allan, P.L., Wright, A.F. Kidney Int. (1992) [Pubmed]
  16. Insulin resistance and hyperinsulinemia are already present in patients with incipient renal disease. Fliser, D., Pacini, G., Engelleiter, R., Kautzky-Willer, A., Prager, R., Franek, E., Ritz, E. Kidney Int. (1998) [Pubmed]
  17. Comparison of Pkd1-targeted mutants reveals that loss of polycystin-1 causes cystogenesis and bone defects. Lu, W., Shen, X., Pavlova, A., Lakkis, M., Ward, C.J., Pritchard, L., Harris, P.C., Genest, D.R., Perez-Atayde, A.R., Zhou, J. Hum. Mol. Genet. (2001) [Pubmed]
  18. Linkage disequilibrium between D16S94 and the locus for adult polycystic kidney disease (PKD1). Elles, R.G. J. Med. Genet. (1992) [Pubmed]
  19. The isolated polycystin-1 cytoplasmic COOH terminus prolongs ATP-stimulated Cl- conductance through increased Ca2+ entry. Wildman, S.S., Hooper, K.M., Turner, C.M., Sham, J.S., Lakatta, E.G., King, B.F., Unwin, R.J., Sutters, M. Am. J. Physiol. Renal Physiol. (2003) [Pubmed]
  20. Autosomal-dominant polycystic kidney disease in the rat. Cowley, B.D., Gudapaty, S., Kraybill, A.L., Barash, B.D., Harding, M.A., Calvet, J.P., Gattone, V.H. Kidney Int. (1993) [Pubmed]
  21. Association of the angiotensin I converting enzyme gene deletion polymorphism with early onset of ESRF in PKD1 adult polycystic kidney disease. Baboolal, K., Ravine, D., Daniels, J., Williams, N., Holmans, P., Coles, G.A., Williams, J.D. Kidney Int. (1997) [Pubmed]
  22. Pathways of caspase-mediated apoptosis in autosomal-dominant polycystic kidney disease (ADPKD). Tao, Y., Kim, J., Stanley, M., He, Z., Faubel, S., Schrier, R.W., Edelstein, C.L. Kidney Int. (2005) [Pubmed]
  23. Decreased synthesis and delayed processing of sulfated glycoproteins by cells from human polycystic kidneys. Carone, F.A., Jin, H., Nakamura, S., Kanwar, Y.S. Lab. Invest. (1993) [Pubmed]
  24. High incidence of common bile duct dilatation in autosomal dominant polycystic kidney disease patients. Ishikawa, I., Chikamoto, E., Nakamura, M., Asaka, M., Tomosugi, N., Yuri, T. Am. J. Kidney Dis. (1996) [Pubmed]
  25. The isolated C-terminus of polycystin-1 promotes increased ATP-stimulated chloride secretion in a collecting duct cell line. Hooper, K.M., Unwin, R.J., Sutters, M. Clin. Sci. (2003) [Pubmed]
  26. Pioglitazone improves the phenotype and molecular defects of a targeted Pkd1 mutant. Muto, S., Aiba, A., Saito, Y., Nakao, K., Nakamura, K., Tomita, K., Kitamura, T., Kurabayashi, M., Nagai, R., Higashihara, E., Harris, P.C., Katsuki, M., Horie, S. Hum. Mol. Genet. (2002) [Pubmed]
  27. Identification of PKDL, a novel polycystic kidney disease 2-like gene whose murine homologue is deleted in mice with kidney and retinal defects. Nomura, H., Turco, A.E., Pei, Y., Kalaydjieva, L., Schiavello, T., Weremowicz, S., Ji, W., Morton, C.C., Meisler, M., Reeders, S.T., Zhou, J. J. Biol. Chem. (1998) [Pubmed]
  28. Autosomal dominant polycystic kidney disease: molecular genetics and molecular pathogenesis. Koptides, M., Deltas, C.C. Hum. Genet. (2000) [Pubmed]
  29. Expression of transforming growth factor alpha and epidermal growth factor receptor in adult polycystic kidney disease. Lee, D.C., Chan, K.W., Chan, S.Y. J. Urol. (1998) [Pubmed]
  30. Polymorphism in the 5' flanking region of the human somatostatin receptor subtype 5. Sasi, R., Puebla, L., Khare, S., Patel, Y.C. Gene (1998) [Pubmed]
  31. The impact of ciclosporin in patients with adult polycystic kidney disease following transplantation. Delaney, V., Sumrani, N., Butt, K.M., Hong, J.H. Nephron (1991) [Pubmed]
  32. Distribution of alpha-integrin subunits in fetal polycystic kidney diseases. Daïkha-Dahmane, F., Narcy, F., Dommergues, M., Lacoste, M., Beziau, A., Gubler, M.C. Pediatr. Nephrol. (1997) [Pubmed]
  33. Imaging of renal cystic diseases. Reznek, R.H., Webb, J.A. Current opinion in radiology. (1991) [Pubmed]
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