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Polycystic kidney disease in the CBA/N immunodeficient mouse
Author(s) -
Rahilly Maeve A.,
Samuel Kay,
Ansell J. D.,
Micklem H. S.,
Fleming S.
Publication year - 1992
Publication title -
the journal of pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.964
H-Index - 184
eISSN - 1096-9896
pISSN - 0022-3417
DOI - 10.1002/path.1711680315
Subject(s) - polycystic kidney disease , pathology , basement membrane , lamina densa , glomerular basement membrane , basal lamina , kidney , nephron , lesion , cyst , biology , epithelium , anatomy , glomerulonephritis , medicine , ultrastructure , endocrinology
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 ceils show subnuciear 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 subnuciear 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.