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Gene Review

Cys1  -  cystin 1

Mus musculus

Synonyms: 2900006B19Rik, AV218859, Ccap, Cilia-associated protein, Cystin-1, ...
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Disease relevance of Cys1


Psychiatry related information on Cys1

  • To determine if the lack of EGF may be a decisive factor in the initiation and/or growth of collecting duct cysts, we administered exogenous EGF (1 microgram/g body wt subcutaneously) daily for Postnatal Days 3-9 (a critical period for collecting duct maturation) to C57BL/6J-cpk mice [5].

High impact information on Cys1

  • Our results indicate that the microtubule cytoskeleton has a central role in the pathogenesis of PKD in cpk mice and that taxol may also be useful in treating human PKD [6].
  • This is associated with abnormal development of primary cilia at the apical pole of the duct cells and with reduced expression of a set of genes involved in polycystic diseases, namely those coding for HNF-1beta and for the cilium-associated proteins polyductin/fibrocystin and cystin [7].
  • Homozygous recessive C57BL/6J (cpk/cpk) mice develop massively enlarged cystic kidneys and die from renal failure shortly after 3 weeks of age [8].
  • The current study was designed: (i) to characterize the cpk-associated biliary disease in affected F(2) homozygotes from intercrosses with either CAST or D2; and (ii) to evaluate focal biliary cysts identified in heterozygotes from a D2-cpk congenic strain [9].
  • Genetic analysis demonstrated loss of heterozygosity at the cpk interval and supports a loss-of-function model for biliary cysts [9].

Chemical compound and disease context of Cys1


Biological context of Cys1


Anatomical context of Cys1

  • Matrix metalloproteinases and TIMPS in cultured C57BL/6J-cpk kidney tubules [3].
  • Between approximately 1 and 3 weeks of age, affected cpk mice develop numerous large cysts in the collecting tubule segment of kidney nephrons [11].
  • We determined in the cpk mouse that the testes similar to the immature ovaries, is also under-developed and arrested at an early developmental stage [15].
  • We conclude that Cys1 and Cys3 are not absolutely necessary for membrane binding or plasma membrane localization [16].
  • Syndet C1S/C3S, with substitutions at the pair Cys1 and Cys3, distributes to the plasma membrane, a Golgi-like compartment, and the cytosol [16].

Associations of Cys1 with chemical compounds

  • This study sought to determine whether the mammalian polycystins are expressed in primary cilia of renal epithelia and whether these proteins co-localize with polaris and cystin, the newly described, cilia-associated protein that is disrupted in the cpk mouse [17].
  • Arrested testis development in the cpk mouse may be the result of abnormal steroid metabolism [15].
  • Among the cysteine replacement analogs, the double mutation of Cys1 and 15 to Ser resulted in significantly increased stability without compromising the mitogenic activity, while Cys to Ser mutations at other positions were either destabilizing or had no effect [18].
  • In addition, the plasma corticosterone levels are higher in a heritable murine model of polycystic kidney disease, cpk mice, in the first postnatal week [19].
  • Aberrant 11beta-hydroxysteroid dehydrogenase-1 activity in the cpk mouse: implications for regulation by the Ke 6 gene [19].

Regulatory relationships of Cys1

  • The overexpression and/or overactivity of the AVP-V2R appears to contribute to the progression of PKD since an AVP-V2R antagonist inhibits cystic renal enlargement in the cpk mouse [20].
  • We report here that the Ke 6 gene and the 11 beta-HSD gene are regulated in the same aberrant pattern in the cpk mouse [21].

Other interactions of Cys1


Analytical, diagnostic and therapeutic context of Cys1


  1. Increased endothelin and endothelin receptor mRNA expression in polycystic kidneys of cpk mice. Nakamura, T., Ebihara, I., Fukui, M., Osada, S., Tomino, Y., Masaki, T., Goto, K., Furuichi, Y., Koide, H. J. Am. Soc. Nephrol. (1993) [Pubmed]
  2. Development of autosomal recessive polycystic kidney disease in BALB/c-cpk/cpk mice. Ricker, J.L., Gattone, V.H., Calvet, J.P., Rankin, C.A. J. Am. Soc. Nephrol. (2000) [Pubmed]
  3. Matrix metalloproteinases and TIMPS in cultured C57BL/6J-cpk kidney tubules. Rankin, C.A., Suzuki, K., Itoh, Y., Ziemer, D.M., Grantham, J.J., Calvet, J.P., Nagase, H. Kidney Int. (1996) [Pubmed]
  4. Structure-function relationships and site of action of apamin, a neurotoxic polypeptide of bee venom with an action on the central nervous system. Vincent, J.P., Schweitz, H., Lazdunski, M. Biochemistry (1975) [Pubmed]
  5. Epidermal growth factor ameliorates autosomal recessive polycystic kidney disease in mice. Gattone, V.H., Lowden, D.A., Cowley, B.D. Dev. Biol. (1995) [Pubmed]
  6. Taxol inhibits progression of congenital polycystic kidney disease. Woo, D.D., Miao, S.Y., Pelayo, J.C., Woolf, A.S. Nature (1994) [Pubmed]
  7. The transcription factor hepatocyte nuclear factor-6 controls the development of pancreatic ducts in the mouse. Pierreux, C.E., Poll, A.V., Kemp, C.R., Clotman, F., Maestro, M.A., Cordi, S., Ferrer, J., Leyns, L., Rousseau, G.G., Lemaigre, F.P. Gastroenterology (2006) [Pubmed]
  8. Elevated c-myc protooncogene expression in autosomal recessive polycystic kidney disease. Cowley, B.D., Smardo, F.L., Grantham, J.J., Calvet, J.P. Proc. Natl. Acad. Sci. U.S.A. (1987) [Pubmed]
  9. Germline and somatic loss of function of the mouse cpk gene causes biliary ductal pathology that is genetically modulated. Guay-Woodford, L.M., Green, W.J., Lindsey, J.R., Beier, D.R. Hum. Mol. Genet. (2000) [Pubmed]
  10. Polycystic kidney and liver disease and corticosterone changes in the cpk mouse. Crocker, J.F., Blecher, S.R., Givner, M.L., McCarthy, S.C. Kidney Int. (1987) [Pubmed]
  11. Defective epidermal growth factor gene expression in mice with polycystic kidney disease. Gattone, V.H., Andrews, G.K., Niu, F.W., Chadwick, L.J., Klein, R.M., Calvet, J.P. Dev. Biol. (1990) [Pubmed]
  12. Tissue inhibitor of matrix metalloproteinase-1 suppresses apoptosis of mouse bone marrow stromal cell line MBA-1. Guo, L.J., Luo, X.H., Xie, H., Zhou, H.D., Yuan, L.Q., Wang, M., Liao, E.Y. Calcif. Tissue Int. (2006) [Pubmed]
  13. Elevated proto-oncogene expression in polycystic kidneys of the C57BL/6J (cpk) mouse. Cowley, B.D., Chadwick, L.J., Grantham, J.J., Calvet, J.P. J. Am. Soc. Nephrol. (1991) [Pubmed]
  14. Differential expression of Cux-1 and p21 in polycystic kidneys from Pkd1 null and cpk mice. Sharma, M., Brantley, J.G., Alcalay, N.I., Zhou, J., Heystek, E., Maser, R.L., Vanden Heuvel, G.B. Kidney Int. (2005) [Pubmed]
  15. Arrested testis development in the cpk mouse may be the result of abnormal steroid metabolism. Aziz, N., Anderson, E., Lee, G.Y., Woo, D.D. Mol. Cell. Endocrinol. (2001) [Pubmed]
  16. Role of the cysteine-rich domain of the t-SNARE component, SYNDET, in membrane binding and subcellular localization. Koticha, D.K., Huddleston, S.J., Witkin, J.W., Baldini, G. J. Biol. Chem. (1999) [Pubmed]
  17. The polycystic kidney disease proteins, polycystin-1, polycystin-2, polaris, and cystin, are co-localized in renal cilia. Yoder, B.K., Hou, X., Guay-Woodford, L.M. J. Am. Soc. Nephrol. (2002) [Pubmed]
  18. Enhanced stability of recombinant keratinocyte growth factor by mutagenesis. Hsu, E., Osslund, T., Nybo, R., Chen, B.L., Kenney, W.C., Morris, C.F., Arakawa, T., Narhi, L.O. Protein Eng. Des. Sel. (2006) [Pubmed]
  19. Aberrant 11beta-hydroxysteroid dehydrogenase-1 activity in the cpk mouse: implications for regulation by the Ke 6 gene. Aziz, N., Brown, D., Lee, W.S., Naray-Fejes-Toth, A. Endocrinology (1996) [Pubmed]
  20. Developmental expression of urine concentration-associated genes and their altered expression in murine infantile-type polycystic kidney disease. Gattone, V.H., Maser, R.L., Tian, C., Rosenberg, J.M., Branden, M.G. Dev. Genet. (1999) [Pubmed]
  21. Coordinate regulation of 11 beta-HSD and Ke 6 genes in cpk mouse: implications for steroid metabolic defect in PKD. Aziz, N., Maxwell, M.M., Brenner, B.M. Am. J. Physiol. (1994) [Pubmed]
  22. Multiorgan mRNA misexpression in murine autosomal recessive polycystic kidney disease. Gattone, V.H., Ricker, J.L., Trambaugh, C.M., Klein, R.M. Kidney Int. (2002) [Pubmed]
  23. 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]
  24. Reduced Pax2 gene dosage increases apoptosis and slows the progression of renal cystic disease. Ostrom, L., Tang, M.J., Gruss, P., Dressler, G.R. Dev. Biol. (2000) [Pubmed]
  25. Localization of overexpressed c-myc mRNA in polycystic kidneys of the cpk mouse. Harding, M.A., Gattone, V.H., Grantham, J.J., Calvet, J.P. Kidney Int. (1992) [Pubmed]
  26. Epidermal growth factor receptor expression is abnormal in murine polycystic kidney. Orellana, S.A., Sweeney, W.E., Neff, C.D., Avner, E.D. Kidney Int. (1995) [Pubmed]
  27. Abnormal tenascin expression in murine autosomal recessive polycystic kidneys. Ojeda, J.L. Nephron (1999) [Pubmed]
  28. Cpk is a novel class of Drosophila PtdIns 3-kinase containing a C2 domain. Molz, L., Chen, Y.W., Hirano, M., Williams, L.T. J. Biol. Chem. (1996) [Pubmed]
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