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MeSH Review

Polycystic Kidney Diseases

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Disease relevance of Polycystic Kidney Diseases


Psychiatry related information on Polycystic Kidney Diseases


High impact information on Polycystic Kidney Diseases

  • The oral-facial-digital type I (OFD1) syndrome (OMIM 311200) is a human developmental disorder; affected individuals have craniofacial and digital abnormalities and, in 15% of cases, polycystic kidney [9].
  • Somatic inactivation of Pkd2 results in polycystic kidney disease [10].
  • Mice heterozygous and homozygous for this mutation, as well as Pkd+/- mice, develop polycystic kidney and liver lesions that are indistinguishable from the human phenotype [10].
  • The polycystic kidney disease 1 (PKD1) gene encodes a novel protein with multiple cell recognition domains [11].
  • Deletion of the TSC2 and PKD1 genes associated with severe infantile polycystic kidney disease--a contiguous gene syndrome [12].

Chemical compound and disease context of Polycystic Kidney Diseases

  • The mean arterial pressures measured in the supine and upright positions and the plasma aldosterone concentrations measured in the upright position were significantly higher in the normotensive patients with polycystic kidney disease than in the normal subjects [13].
  • It is thought that cysts in polycystic kidneys originate from nephron segments and function in a manner similar to the segment or origin [14].
  • Cystin, a novel cilia-associated protein, is disrupted in the cpk mouse model of polycystic kidney disease [15].
  • Cyclic AMP concentration was higher in distal than in proximal cysts (663 vs 6.0 pmol/L). mRNA of HGF and Met were co-expressed in cyst walls from cases with polycystic kidney disease [16].
  • Polycystic kidney disease and angiotensin-converting enzyme inhibitors [17].

Biological context of Polycystic Kidney Diseases


Anatomical context of Polycystic Kidney Diseases


Gene context of Polycystic Kidney Diseases


Analytical, diagnostic and therapeutic context of Polycystic Kidney Diseases

  • These results show that both dietary protein source and level significantly affect polycystic kidney disease in pcy animals, with the effects of dietary soy protein being most pronounced in female animals fed the low protein diets and the effects of protein reduction being most pronounced in animals fed soy protein-based diets [31].
  • The presence of messenger RNA for the mouse homologue of the polycystic kidney disease 1 gene (PKD1) was demonstrated by reverse transcription-polymerase chain reaction (RT-PCR) methods in mouse embryo messenger RNA [32].
  • We used a human renin antiserum and an immunoperoxidase method to investigate the distribution of renin-containing cells in 19 adult polycystic kidneys: 9 autopsy and 10 nephrectomy cases [33].
  • Those patients with both PCLD and polycystic kidney disease who are not dialysis dependent can be managed for several years with isolated liver transplantation and then receive kidney transplantation if needed [34].
  • To study the role of TGF alpha in cyst formation, we analyzed nine anatomically diagnosed adult polycystic kidneys and four normal kidneys using immunohistochemistry [35].


  1. Apoptosis and loss of renal tissue in polycystic kidney diseases. Woo, D. N. Engl. J. Med. (1995) [Pubmed]
  2. Genetic identification of two major modifier loci of polycystic kidney disease progression in pcy mice. Woo, D.D., Nguyen, D.K., Khatibi, N., Olsen, P. J. Clin. Invest. (1997) [Pubmed]
  3. Reversible renal failure associated with angiotensin-converting enzyme inhibitors in polycystic kidney disease. Chapman, A.B., Gabow, P.A., Schrier, R.W. Ann. Intern. Med. (1991) [Pubmed]
  4. PKHD1 protein encoded by the gene for autosomal recessive polycystic kidney disease associates with basal bodies and primary cilia in renal epithelial cells. Zhang, M.Z., Mai, W., Li, C., Cho, S.Y., Hao, C., Moeckel, G., Zhao, R., Kim, I., Wang, J., Xiong, H., Wang, H., Sato, Y., Wu, Y., Nakanuma, Y., Lilova, M., Pei, Y., Harris, R.C., Li, S., Coffey, R.J., Sun, L., Wu, D., Chen, X.Z., Breyer, M.D., Zhao, Z.J., McKanna, J.A., Wu, G. Proc. Natl. Acad. Sci. U.S.A. (2004) [Pubmed]
  5. Polycystic kidney disease in SBM transgenic mice: role of c-myc in disease induction and progression. Trudel, M., Barisoni, L., Lanoix, J., D'Agati, V. Am. J. Pathol. (1998) [Pubmed]
  6. Pancreatitis in patients with end-stage renal disease. Padilla, B., Pollak, V.E., Pesce, A., Kant, K.S., Gilinsky, N.H., Deddens, J.A. Medicine (Baltimore) (1994) [Pubmed]
  7. Human ARHGDIG, a GDP-dissociation inhibitor for Rho proteins: genomic structure, sequence, expression analysis, and mapping to chromosome 16p13.3. Adra, C.N., Iyengar, A.R., Syed, F.A., Kanaan, I.N., Rilo, H.L., Yu, W., Kheraj, R., Lin, S.R., Horiuchi, T., Khan, S., Weremowicz, S., Lim, B., Morton, C.C., Higgs, D.R. Genomics (1998) [Pubmed]
  8. Strain difference in expression of the adult-type polycystic kidney disease gene, pcy, in the mouse. Nagao, S., Hibino, T., Koyama, Y., Marunouchi, T., Konishi, H., Takahashi, H. Jikken Dobutsu (1991) [Pubmed]
  9. Oral-facial-digital type I protein is required for primary cilia formation and left-right axis specification. Ferrante, M.I., Zullo, A., Barra, A., Bimonte, S., Messaddeq, N., Studer, M., Dollé, P., Franco, B. Nat. Genet. (2006) [Pubmed]
  10. 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]
  11. The polycystic kidney disease 1 (PKD1) gene encodes a novel protein with multiple cell recognition domains. Hughes, J., Ward, C.J., Peral, B., Aspinwall, R., Clark, K., San Millán, J.L., Gamble, V., Harris, P.C. Nat. Genet. (1995) [Pubmed]
  12. Deletion of the TSC2 and PKD1 genes associated with severe infantile polycystic kidney disease--a contiguous gene syndrome. Brook-Carter, P.T., Peral, B., Ward, C.J., Thompson, P., Hughes, J., Maheshwar, M.M., Nellist, M., Gamble, V., Harris, P.C., Sampson, J.R. Nat. Genet. (1994) [Pubmed]
  13. The renin-angiotensin-aldosterone system and autosomal dominant polycystic kidney disease. Chapman, A.B., Johnson, A., Gabow, P.A., Schrier, R.W. N. Engl. J. Med. (1990) [Pubmed]
  14. In vitro function of cyst epithelium from human polycystic kidney. Perrone, R.D. J. Clin. Invest. (1985) [Pubmed]
  15. Cystin, a novel cilia-associated protein, is disrupted in the cpk mouse model of polycystic kidney disease. Hou, X., Mrug, M., Yoder, B.K., Lefkowitz, E.J., Kremmidiotis, G., D'Eustachio, P., Beier, D.R., Guay-Woodford, L.M. J. Clin. Invest. (2002) [Pubmed]
  16. Mediation of renal cyst formation by hepatocyte growth factor. Horie, S., Higashihara, E., Nutahara, K., Mikami, Y., Okubo, A., Kano, M., Kawabe, K. Lancet (1994) [Pubmed]
  17. Polycystic kidney disease and angiotensin-converting enzyme inhibitors. Bursztyn, M. Ann. Intern. Med. (1992) [Pubmed]
  18. Rapid genetic analysis of families with polycystic kidney disease 1 by means of a microsatellite marker. Harris, P.C., Thomas, S., Ratcliffe, P.J., Breuning, M.H., Coto, E., Lopez-Larrea, C. Lancet (1991) [Pubmed]
  19. Mutations in a NIMA-related kinase gene, Nek1, cause pleiotropic effects including a progressive polycystic kidney disease in mice. Upadhya, P., Birkenmeier, E.H., Birkenmeier, C.S., Barker, J.E. Proc. Natl. Acad. Sci. U.S.A. (2000) [Pubmed]
  20. Evidence of linkage disequilibrium in the Spanish polycystic kidney disease I population. Peral, B., Ward, C.J., San Millán, J.L., Thomas, S., Stallings, R.L., Moreno, F., Harris, P.C. Am. J. Hum. Genet. (1994) [Pubmed]
  21. A translation frameshift mutation induced by a cytosine insertion in the polycystic kidney disease 2 gene (PDK2). Xenophontos, S., Constantinides, R., Hayashi, T., Mochizuki, T., Somlo, S., Pierides, A., Deltas, C.C. Hum. Mol. Genet. (1997) [Pubmed]
  22. Chlamydomonas IFT88 and its mouse homologue, polycystic kidney disease gene tg737, are required for assembly of cilia and flagella. Pazour, G.J., Dickert, B.L., Vucica, Y., Seeley, E.S., Rosenbaum, J.L., Witman, G.B., Cole, D.G. J. Cell Biol. (2000) [Pubmed]
  23. Polycystin, the polycystic kidney disease 1 protein, is expressed by epithelial cells in fetal, adult, and polycystic kidney. Ward, C.J., Turley, H., Ong, A.C., Comley, M., Biddolph, S., Chetty, R., Ratcliffe, P.J., Gattner, K., Harris, P.C. Proc. Natl. Acad. Sci. U.S.A. (1996) [Pubmed]
  24. The polycystic kidney disease protein PKD2 interacts with Hax-1, a protein associated with the actin cytoskeleton. Gallagher, A.R., Cedzich, A., Gretz, N., Somlo, S., Witzgall, R. Proc. Natl. Acad. Sci. U.S.A. (2000) [Pubmed]
  25. Apical plasma membrane mispolarization of NaK-ATPase in polycystic kidney disease epithelia is associated with aberrant expression of the beta2 isoform. Wilson, P.D., Devuyst, O., Li, X., Gatti, L., Falkenstein, D., Robinson, S., Fambrough, D., Burrow, C.R. Am. J. Pathol. (2000) [Pubmed]
  26. The SGP-2 gene is developmentally regulated in the mouse kidney and abnormally expressed in collecting duct cysts in polycystic kidney disease. Harding, M.A., Chadwick, L.J., Gattone, V.H., Calvet, J.P. Dev. Biol. (1991) [Pubmed]
  27. PKD2, a gene for polycystic kidney disease that encodes an integral membrane protein. Mochizuki, T., Wu, G., Hayashi, T., Xenophontos, S.L., Veldhuisen, B., Saris, J.J., Reynolds, D.M., Cai, Y., Gabow, P.A., Pierides, A., Kimberling, W.J., Breuning, M.H., Deltas, C.C., Peters, D.J., Somlo, S. Science (1996) [Pubmed]
  28. Screening the 3' region of the polycystic kidney disease 1 (PKD1) gene reveals six novel mutations. Peral, B., San Millán, J.L., Ong, A.C., Gamble, V., Ward, C.J., Strong, C., Harris, P.C. Am. J. Hum. Genet. (1996) [Pubmed]
  29. Mutations in PRKCSH cause isolated autosomal dominant polycystic liver disease. Li, A., Davila, S., Furu, L., Qian, Q., Tian, X., Kamath, P.S., King, B.F., Torres, V.E., Somlo, S. Am. J. Hum. Genet. (2003) [Pubmed]
  30. The polycystic kidney disease 1 gene product mediates protein kinase C alpha-dependent and c-Jun N-terminal kinase-dependent activation of the transcription factor AP-1. Arnould, T., Kim, E., Tsiokas, L., Jochimsen, F., Grüning, W., Chang, J.D., Walz, G. J. Biol. Chem. (1998) [Pubmed]
  31. Dietary soy protein effects on inherited polycystic kidney disease are influenced by gender and protein level. Aukema, H.M., Housini, I., Rawling, J.M. J. Am. Soc. Nephrol. (1999) [Pubmed]
  32. Expression of polycystin in mouse metanephros and extra-metanephric tissues. Griffin, M.D., O'Sullivan, D.A., Torres, V.E., Grande, J.P., Kanwar, Y.S., Kumar, R. Kidney Int. (1997) [Pubmed]
  33. The anatomy of the renin-secreting cell in adult polycystic kidney disease. Graham, P.C., Lindop, G.B. Kidney Int. (1988) [Pubmed]
  34. Liver and kidney transplantation for polycystic disease. Jeyarajah, D.R., Gonwa, T.A., Testa, G., Abbasoglu, O., Goldstein, R., Husberg, B.S., Levy, M.F., Klintmalm, G.B. Transplantation (1998) [Pubmed]
  35. 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]
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