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Published on: June 23, 2015
An Overview of In Vivo and In Vitro Models for Autosomal Dominant Polycystic Kidney Disease: A Journey from 3D-Cysts
Svenja Koslowski1,2, Camille Latapy2, Pierrïck Auvray2
1Perha Pharmaceuticals & ManRos Therapeutics, Centre de Perharidy, 29680 Roscoff, Bretagne, France.
Abstract:
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inheritable cause of end stage renal disease and, as of today, only a single moderately effective treatment is available for patients. Even though ADPKD research has made huge progress over the last decades, the precise disease mechanisms remain elusive. However, a wide variety of cellular and animal models have been developed to decipher the pathophysiological mechanisms and related pathways underlying the disease. As none of these models perfectly recapitulates the complexity of the human disease, the aim of this review is to give an overview of the main tools currently available to ADPKD researchers, as well as their main advantages and limitations.
Insights
Autosomal dominant polycystic kidney disease (ADPKD) research is advancing, but disease mechanisms remain unclear. This review overviews current ADPKD research models, detailing their strengths and weaknesses for understanding this kidney disease.
Area of Science:
- Nephrology
- Genetics
- Translational Medicine
Background:
- Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited cause of end-stage renal disease.
- Current treatments for ADPKD are limited, with only one moderately effective option available.
- Despite significant research progress, the exact pathophysiological mechanisms of ADPKD are not fully understood.
Purpose of the Study:
- To provide a comprehensive overview of the various cellular and animal models used in ADPKD research.
- To critically evaluate the advantages and limitations of each model in deciphering ADPKD's complex mechanisms.
- To guide researchers in selecting appropriate tools for studying ADPKD.
Main Methods:
- Review of existing literature on ADPKD models.
- Analysis of commonly utilized cellular models (e.g., cell cultures, organoids).
- Evaluation of prevalent animal models (e.g., mouse, rat, zebrafish models).
Main Results:
- Various models exist, including cell cultures, organoids, and animal models, each offering unique insights.
- No single model perfectly replicates the complexity of human ADPKD.
- Models differ in their ability to recapitulate specific aspects of ADPKD, such as cyst formation and progression.
Conclusions:
- Understanding the advantages and limitations of each ADPKD model is crucial for effective research.
- The selection of an appropriate model depends on the specific research question being addressed.
- Continued development and refinement of ADPKD models are necessary to advance therapeutic strategies.
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