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Published on: September 1, 2015
The Role of PEC Progenitors in ADPKD Progression
Daniele Lodi1, Giulia Ligabue, Valentina Lupo
1Division of Nephrology, Modena Polyclinic, Modena, Italy.
Insights
Stem/progenitor cells CD133(+)CD24(+) are involved in Autosomal dominant polycystic kidney disease (ADPKD) progression. These cells are more abundant in ADPKD kidneys, suggesting a role in pathogenesis.
Area of Science:
- Nephrology
- Stem Cell Biology
- Pathogenesis of Kidney Diseases
Background:
- Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive kidney cyst development.
- Renal tubules normally repair damage using stem/progenitor cells (CD133(+)CD24(+)).
- Insufficient repair in ADPKD suggests a potential role for these stem cells in disease progression.
Purpose of the Study:
- To investigate the localization and involvement of CD133(+)CD24(+) cells in ADPKD progression.
- To determine if these progenitor cells contribute to the pathogenesis of ADPKD.
Main Methods:
- Analysis of normal and ADPKD kidney tissues.
- Confocal microscopy and immunoblotting to assess CD133 and CD24 expression.
- Quantification of CD133(+)CD24(+) cells in renal tissues.
Main Results:
- CD133 and CD24 expression was observed in a subset of epithelial cells in both normal and ADPKD kidneys.
- These cells are located in Bowman's capsule and the luminal side of tubules.
- CD133(+)CD24(+) cells were significantly more represented in ADPKD tubules and healthy glomeruli.
- ADPKD cysts also expressed CD133 and CD24.
Conclusions:
- Renal epithelial progenitors (CD133(+)CD24(+)) are involved in ADPKD pathogenesis.
- Further research is needed to clarify their precise role.
- Targeting these cells may offer a strategy for disease management and stabilization.
Background And Objectives:
Autosomal dominant polycystic kidney disease is a pathology mainly characterized by the progressive development and enlargement of cysts in each kidneys. Such as many adult epithelial tissue, renal tubule replaces damaged or death cells through the presence of stem/progenitor cells CD133(+)CD24(+) Obviously, in ADPKD the repair of damages is insufficient to block the disease, but renal stem cells could have a role in the pathology. In this study we investigate the localization and the involvement of cells CD133(+)CD24(+) in ADPKD progression.
Methods And Results:
Two normal kidneys and two ADPKD kidneys were examined. CD133 and CD24 expression was investigated by confocal microscopy and immunoblotting. Renal tissue and cells were analyzed. CD133 and CD24 have the same localization in ADPKD tissues and in normal kidneys: a subset of epithelial cells (PEC) of Bowman' s capsule and luminal side of tubules. It is interesting that CD133(+) CD24(+) cells are statistically more represented in ADPKD tubules (p< 0.001) and in healthy glomeruli (p= 0.0016). Cysts express CD133 and CD24.
Conclusions:
Renal epithelial progenitors demonstrate to be involved in ADPKD pathogenesis but their role will have to be clarified and possibly managed to obtain improvement, or at least stabilization, of disease.
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