Overexpression of innate immune response genes in a model of recessive polycystic kidney disease

Academic Article


  • Defects in the primary cilium/basal body complex of renal tubular cells cause polycystic kidney disease (PKD). To uncover pathways associated with disease progression, we determined the kidney transcriptome of 10-day-old severely and mildly affected cpk mice, a model of recessive PKD. In the severe phenotype, the most highly expressed genes were those associated with the innate immune response including many macrophage markers, particularly those associated with a profibrotic alternative activation pathway. Additionally, gene expression of macrophage activators was dominated by the complement system factors including the central complement component 3. Additional studies confirmed increased complement component 3 protein levels in both cystic and non-cystic epithelia in the kidneys of cpk compared to wild-type mice. We also found elevated complement component 3 activation in two other mouse-recessive models and human-recessive PKD. Our results suggest that abnormal complement component 3 activation is a key element of progression in PKD. © 2008 International Society of Nephrology.
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    Digital Object Identifier (doi)

    Author List

  • Mrug M; Zhou J; Woo Y; Cui X; Szalai AJ; Novak J; Churchill GA; Guay-Woodford LM
  • Start Page

  • 63
  • End Page

  • 76
  • Volume

  • 73
  • Issue

  • 1