Polycystic kidney disease in the CBA/N immunodeficient mouse
| Autor: | Stewart Fleming, J. D. Ansell, Kay Samuel, H. S. Micklem, Maeve A. Rahilly |
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| Rok vydání: | 1992 |
| Předmět: |
Basement membrane
Polycystic Kidney Diseases Kidney Pathology medicine.medical_specialty Age Factors Immunologic Deficiency Syndromes Anatomy Biology medicine.disease Mice Mutant Strains Epithelium Pathology and Forensic Medicine Lesion Disease Models Animal Mice medicine.anatomical_structure medicine Polycystic kidney disease Animals Lamina densa Cyst medicine.symptom Immunodeficient Mouse |
| Zdroj: | The Journal of Pathology. 168:335-342 |
| ISSN: | 1096-9896 0022-3417 |
| Popis: | We describe a polycystic lesion of the kidney in the CBA/N mouse with an X-linked recessive immunodeficient syndrome. There is progressive cystic dilatation affecting all parts of the nephron. The cyst lining is composed of a single layered epithelium with focal nuclear crowding and the formation of micropapillary structures. The cystic epithelial cells show subnuclear vacuolation. Focal basement membrane thickening is also a feature. There is no significant inflammatory infiltrate present within these kidneys. Electron microscopic examination reveals that the subnuclear vacuolation is due to loss of the membrane infoldings at the basal pole of the epithelial cell with fluid accumulation within the extracellular space. The basement membrane thickening is due to expansion of the lamina densa. These changes are not present at birth but develop progressively with age. The finding of a polycystic kidney lesion in these mice offers an opportunity to investigate the relationship between the immune system and renal cyst formation. |
| Databáze: | OpenAIRE |
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