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Updated: Jun 10, 2026
Comparative Proteomic Analysis of Whole Kidney, Medulla, and Cortical Tubules in Diabetic Pathogenesis of Kidney Injury in Mice
Published on: May 2, 2025
Differential expression of renal proteins in a rodent model of Meckel syndrome
Stephen B Mason1, Xianyin Lai, Heather N Ringham
1Department of Anatomy and Cell Biology, Indiana University School of Medicine, Indianapolis, USA.
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.
Insights
Researchers identified misexpressed proteins in a rat model of Meckel syndrome (MKS). These protein changes may serve as biomarkers for staging the progression of kidney cysts in MKS and other polycystic kidney diseases.
Area of Science:
- Biochemistry
- Genetics
- Nephrology
Background:
- Meckel syndrome (MKS) is a fatal autosomal recessive disorder characterized by severe kidney cyst formation.
- The Wpk rat serves as a model for human MKS3 gene disease, exhibiting prominent renal cystic pathology.
Purpose of the Study:
- To identify potential biomarkers for staging renal cystic progression in Meckel syndrome.
- To evaluate renal protein misexpression in the Wpk rat model of MKS.
Main Methods:
- Proteomic analysis including two-dimensional gel electrophoresis and LC-MS/MS was employed.
- Western blot analysis was used to confirm differential protein expression.
Main Results:
- Proteomic analysis identified 76 differentially abundant proteins between early and late stages of MKS renal cystic disease.
- Galectin-1 and vimentin were found to be overexpressed, consistent with findings in other ciliopathic models.
- Polycystin 1 & 2 and fibrocystin, key polycystic kidney disease (PKD) proteins, were also differentially expressed.
Conclusions:
- Misexpressed proteins implicated in other cystic kidney diseases were identified in the Wpk rat model.
- Significant protein overexpression and underexpression were observed in late-stage MKS renal disease.
- Differential protein expression patterns may serve as valuable biomarkers for diagnosing and monitoring cystic kidney disease progression.
