論文 - 詳細
| RRC ID | 266 |
|---|---|
| 著者 | Mochizuki E, Fukuta K, Tada T, Harada T, Watanabe N, Matsuo S, Hashimoto H, Ozato K, Wakamatsu Y. |
| タイトル | Fish mesonephric model of polycystic kidney disease in medaka (Oryzias latipes) pc mutant. |
| ジャーナル | 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. |
| 巻・号 | 68(1) |
| ページ | 23-34 |
| 公開日 | 2005-7-1 |
| 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 | 8.945 |
| 引用数 | 25 |
| WOS 分野 | UROLOGY & NEPHROLOGY |
| リソース情報 | |
| メダカ | ci (MT15) pc (MT83) STII-pc (MT850) |