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Polycystic kidneys caused by sustained expression of Cux1 isoform p75

Chantal Cadieux1, Ryoko Harada, Marilène Paquet

  • 1Department of Biochemistry, McGill University, Montreal H3G 1Y6 Canada.

The Journal of Biological Chemistry
|March 22, 2008
PubMed

Insights

The shortest Cux1 isoform (p75-Cux1) drives polycystic kidney disease development by altering gene expression and promoting cell proliferation. This finding implicates p75-Cux1 as a key factor in renal cyst formation.

Area of Science:

  • Molecular Biology
  • Genetics
  • Nephrology

Background:

  • The transcriptional regulator Cux1 (CDP, Cutl1) is implicated in polycystic kidney disease (PKD).
  • The p75-Cux1 isoform, transcribed from an alternative promoter, is deregulated in Pkd1 mutant mouse kidneys.

Purpose of the Study:

  • To investigate the role of the p75-Cux1 isoform in polycystic kidney disease pathogenesis.
  • To determine the molecular mechanisms by which p75-Cux1 contributes to renal cystogenesis.

Main Methods:

  • Generation of transgenic mice expressing p75-CUX1 in renal tissues.
  • Histological and marker analysis of polycystic kidneys.
  • Chromatin affinity purification to assess promoter interactions.

Main Results:

  • Transgenic mice expressing p75-CUX1 developed polycystic kidneys with variable severity.
  • p75-CUX1-induced polycystic kidneys exhibited upregulated c-myc and downregulated p27.
  • Cux1 directly interacted with c-myc and p27 promoters, leading to increased cilia length and epithelial cell proliferation.

Conclusions:

  • The shortest isoform of CUX1 (p75-Cux1) plays a significant role in the development of polycystic kidney disease.
  • p75-Cux1 promotes cystogenesis through modulation of c-myc and p27 expression, impacting cell proliferation and cilia length.

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