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Published on: June 23, 2015
The Han:SPRD Rat: A Preclinical Model of Polycystic Kidney Disease
Ioannis Kofotolios1,2, Michael J Bonios3, Markos Adamopoulos2
1Clinic of Nephrology and Renal Tranplantation, Laiko Hospital, Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece.
Insights
The Han:SPRD rat model, with an AnkS6 mutation, mimics human Autosomal Dominant Polycystic Kidney Disease (ADPKD). This model is crucial for understanding ADPKD pathogenesis and testing new therapies.
Area of Science:
- Nephrology
- Genetics
- Animal Models
Background:
- Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most common inherited kidney disease, often leading to kidney failure.
- Animal models are vital for studying ADPKD mechanisms and developing treatments.
- The Han:SPRD rat harbors an AnkS6 gene mutation, causing polycystic kidneys.
Purpose of the Study:
- To review the utility of the Han:SPRD rat model for Autosomal Dominant Polycystic Kidney Disease (ADPKD) research.
- To highlight the phenotypic parallels between the Han:SPRD rat and human ADPKD.
- To emphasize the model's significance in preclinical studies and therapeutic development.
Main Methods:
- Characterization of the Han:SPRD rat model with an R823W mutation in the AnkS6 gene.
- Analysis of cyst formation and kidney enlargement in the rat model.
- Investigation of the mutated protein, Samcystin, in renal tubular epithelial cells and cilia.
Main Results:
- The Han:SPRD rat exhibits kidney enlargement and cyst formation, mirroring human ADPKD.
- The mutated Samcystin protein localizes to cilia and is implicated in cystogenesis.
- Homozygous AnkS6 mutation results in end-stage renal disease and mortality.
Conclusions:
- The Han:SPRD rat is a valuable preclinical model for Autosomal Dominant Polycystic Kidney Disease (ADPKD).
- This model aids in elucidating ADPKD pathogenesis and evaluating novel therapeutic strategies.
- Further research using this model can accelerate the development of effective ADPKD treatments.
Abstract:
Autosomal Dominant Polycystic Kidney Disease (ADPKD) stands as the most prevalent hereditary renal disorder in humans, ultimately culminating in end-stage kidney disease. Animal models carrying mutations associated with polycystic kidney disease have played an important role in the advancement of ADPKD research. The Han:SPRD rat model, carrying an R823W mutation in the Anks6 gene, is characterized by cyst formation and kidney enlargement. The mutated protein, named Samcystin, is localized in cilia of tubular epithelial cells and seems to be involved in cystogenesis. The homozygous Anks6 mutation leads to end-stage renal disease and death, making it a critical factor in kidney development and function. This review explores the utility of the Han:SPRD rat model, highlighting its phenotypic similarity to human ADPKD. Specifically, we discuss its role in preclinical trials and its importance for investigating the pathogenesis of the disease and developing new therapeutic approaches.
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