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

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Abstract

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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Mrug, M., Zhou, J., Woo, Y., Cui, X., Szalai, A. J., Novak, J., … Guay-Woodford, L. M. (2008). Overexpression of innate immune response genes in a model of recessive polycystic kidney disease. Kidney International, 73(1), 63–76. https://doi.org/10.1038/sj.ki.5002627

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