Human Cell

, Volume 16, Issue 2, pp 65–72

Identification of a new target molecule for a cascade therapy of polycystic kidney

  • Noriyuki Yoshida
  • Yoshihisa Yano
  • Atsushi Yoshiki
  • Munehisa Ueno
  • Nobuhiro Deguchi
  • Shinji Hirotsune
Review

Abstract

Autosomal dominant polycystic kidney disease is a systemic disorder that primary affects the kidney which is characterized by the formation of fluid-filled cysts in both kidneys that leads to progressive renal failure. Mutated genes, polycystin-1 and polycystin-2, are identified, and evidence has emerged that polycystins are ion channels or regulators of ion channels. In spite of extensive characterization of polycystins, how polycystin channel signaling may be involved in cyst formation in ADPKD is still unclear. We found a mutant mouse which exhibits polycystic kidney and bone deformity in the course of making a transgenic mouse carrying the Drosophila sex-lethal gene. We identifed a mutated gene Makorin1 by positional cloning. Makorin1 carries a typical RING-finger motif, suggesting that Makorin1 belongs to ubiquitinase E3 family. Makorin1 would open a new avenue to understand pathogenesis of polycystic kidney, and become a new therapeutic target of polycystic kidney.

Keywords

Polycystic Kidney Polycystin Makorin1 Mouse Model 

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Copyright information

© Society and Springer Japan 2003

Authors and Affiliations

  • Noriyuki Yoshida
    • 1
  • Yoshihisa Yano
    • 2
  • Atsushi Yoshiki
    • 3
  • Munehisa Ueno
    • 4
  • Nobuhiro Deguchi
    • 4
  • Shinji Hirotsune
    • 2
  1. 1.Division of Chemical BiologyJapan
  2. 2.Division of Genetic Disease Research, Osaka CityUniversity Graduate School of MedicineOsakaJapan
  3. 3.Experimental Animal Division, Department of Biological Systems BioResource CenterRIKEN Tsukuba InstituteIbarakiJapan
  4. 4.Department of Urology Kidney Disease CenterSaitama Medical School SaitamaJapan

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