Differential expression of renal proteins in a rodent model of meckel syndrome

Stephen B. Mason, Xianyin Lai, Heather N. Ringham, Robert L. Bacallao, Peter C. Harris, Frank A. Witzmann, Vincent H. Gattone

Research output: Contribution to journalArticlepeer-review

4 Scopus citations


Background: Meckel syndrome (MKS) is a fatal autosomal recessive condition with prominent renal cystic pathology. Renal protein misexpression was evaluated in the Wpk rat model of human MKS3 gene disease to identify biomarkers for the staging of renal cystic progression. Methods: Misexpressed proteins were compared between early and late stages of MKS renal cystic disease using proteomic analysis (two-dimensional gel electrophoresis with LC-MS/MS identification) followed by Western blot analysis. Results: A proteomic analysis identified 76 proteins with statistically different, normalized abundance in at least one group. Subsequently, Western blot was used to confirm differential expression in several of these and polycystic kidney disease (PKD)-associated proteins. Galectin-1 and vimentin were identified as overexpressed proteins, which have been previously found in the jck mouse model of nephronophthisis 9. Ciliopathic PKD proteins, polycystins 1 & 2, and fibrocystin were also differentially expressed in Wpk kidney. Conclusion: In the Wpk rat, misexpressed proteins were identified that were also implicated in other forms of cystic disease. Numerous proteins were either over- or underexpressed in late-stage disease. Differences in protein expression may serve as biomarkers of cystic disease and its progression.

Original languageEnglish (US)
Pages (from-to)e31-e38
JournalNephron - Experimental Nephrology
Issue number2
StatePublished - Jan 2011


  • Biomarker
  • Polycystic kidney
  • Proteomics

ASJC Scopus subject areas

  • Physiology
  • Genetics
  • Nephrology


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