| Autor: |
Kofotolios I; Clinic of Nephrology and Renal Tranplantation, Laiko Hospital, Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece.; Center of Basic Research, Biomedical Research Foundation, Academy of Athens, 11527 Athens, Greece., Bonios MJ; Heart Failure and Transplant Unit, Onassis Cardiac Surgery Center, 17674 Athens, Greece., Adamopoulos M; Center of Basic Research, Biomedical Research Foundation, Academy of Athens, 11527 Athens, Greece., Mourouzis I; Department of Pharmacology, National and Kapodistrian University of Athens, 11527 Athens, Greece., Filippatos G; Department of Cardiology, Attikon University Hospital, Medical School, National and Kapodistrian University of Athens, 12462 Athens, Greece., Boletis JN; Clinic of Nephrology and Renal Tranplantation, Laiko Hospital, Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece., Marinaki S; Clinic of Nephrology and Renal Tranplantation, Laiko Hospital, Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece., Mavroidis M; Center of Basic Research, Biomedical Research Foundation, Academy of Athens, 11527 Athens, Greece. |
| Abstrakt: |
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. |
