Reference - Detail
|Author||Mochizuki E, Fukuta K, Tada T, Harada T, Watanabe N, Matsuo S, Hashimoto H, Ozato K, Wakamatsu Y.|
|Title||Fish mesonephric model of polycystic kidney disease in medaka (Oryzias latipes) pc mutant.|
BACKGROUND:Polycystic kidney disease (PKD) is a common hereditary disease. A number of murine and zebrafish mutants have been generated and used for the study of PKD as metanephric and pronephric models, respectively. Here, we report a medaka (Oryzias latipes) mutant that develops numerous cysts in the kidney in adulthood fish in an autosomal-recessive manner as a mesonephric model of PKD.
METHODS:The phenotypes of the medaka pc mutant were described in terms of morphologic, histologic, and ultrastructural features. The pc see-through stock was produced by crossing a pc mutant and a fish from the see-through stock and used for observing the kidney through the transparent body wall of a live fish.
RESULTS:The mutant developed bilateral massive enlargement of the kidney in adulthood. They sexually matured normally within 2 months of age and died within 6 months of age. The affected kidney was occupied by numerous, fluid-filled cysts, which were lined by attenuated squamous epithelial cells. Developmentally, cystic formation began in the pronephros in 10-day-old fry and in the mesonephros in 20-day-old fry at the microscopic level. The pc see-through stock was useful in observing disease progression in live fish.
CONCLUSION:The kidney disorder that develops in the medaka pc mutant is a mesonephric counterpart of PKD, particularly an autosomal-dominant PKD, based on its morphologic, histologic, and ultrastructural features, and slow progression.
|MeSH||Animals Disease Models, Animal* Female Genes, Dominant Kidney Tubules / abnormalities Kidney Tubules / pathology Kidney Tubules / ultrastructure Male Mesonephros / pathology* Mesonephros / ultrastructure Microscopy, Electron, Scanning Microscopy, Electron, Transmission Mutation Nephrons / abnormalities Nephrons / pathology Nephrons / ultrastructure Organ Size Oryzias / abnormalities Oryzias / genetics* Phenotype Polycystic Kidney Diseases / genetics* Polycystic Kidney Diseases / pathology*|
|WOS Category||UROLOGY & NEPHROLOGY|
|Medaka||ci (MT15) pc (MT83) STII-pc (MT850)|