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Publication : Gene expression in early and progression phases of autosomal dominant polycystic kidney disease.

First Author  Chen WC Year  2008
Journal  BMC Res Notes Volume  1
Pages  131 PubMed ID  19099603
Mgi Jnum  J:355851 Mgi Id  MGI:7761943
Doi  10.1186/1756-0500-1-131 Citation  Chen WC, et al. (2008) Gene expression in early and progression phases of autosomal dominant polycystic kidney disease. BMC Res Notes 1:131
abstractText  BACKGROUND: Little is known about the genes involved in the initial cyst formation and disease progression in autosomal dominant polycystic kidney disease (ADPKD); however, such knowledge is necessary to explore therapeutic avenues for this common inherited kidney disease. FINDINGS: To uncover the genetic determinants and molecular mechanisms of ADPKD, we analyzed 4-point time-series DNA microarrays from Pkd1L3/L3 mice to generate high resolution gene expression profiles at different stages of disease progression. We found different characteristic gene expression signatures in the kidneys of Pkd1L3/L3 mice compared to age-matched controls during the initial phase of the disease. By postnatal week 1, the Pkd1L3/L3 kidney already had a distinctive gene expression pattern different from the corresponding normal controls. CONCLUSION: The genes differentially expressed, either induced or repressed, in ADPKD are important in immune defense, cell structure and motility, cellular proliferation, apoptosis and metabolic processes, and include members of three pathways (Wnt, Notch, and BMP) involved in morphogenetic signaling. Further analysis of the gene expression profiles from the early stage of cystogenesis to end stage disease identified a possible gene network involved in the pathogenesis of ADPKD.
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