| First Author | Mrug M | Year | 2008 |
| Journal | Kidney Int | Volume | 73 |
| Issue | 1 | Pages | 63-76 |
| PubMed ID | 17960140 | Mgi Jnum | J:135367 |
| Mgi Id | MGI:3793522 | Doi | 10.1038/sj.ki.5002627 |
| Citation | Mrug M, et al. (2008) Overexpression of innate immune response genes in a model of recessive polycystic kidney disease. Kidney Int 73(1):63-76 |
| abstractText | 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. |
