RRC ID 266
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.
Journal Kidney Int.
Abstract 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.
Volume 68(1)
Pages 23-34
Published 2005-7
DOI 10.1111/j.1523-1755.2005.00378.x
PII S0085-2538(15)50811-9
PMID 15954893
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*
IF 8.429
Times Cited 20
Medaka ci (MT15) pc (MT83) STII-pc (MT850)