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Pronephric mutants and cystic disease Kidney cystic disease is characterized by enlarged, swollen, fluid-filled kidney tubule lumens and stretched distended epithelia. We have isolated eighteen independent recessive mutations affecting pronephric development. The visible unifying phenotype of all the mutants is the appearance of fluid filled cysts in the region of the pronephros. Our recent work has shown that this phenotype can arise as a result of failure to clear fluid from the kidney. In particular, we have shown that beating cilia act as fluid pumps to drive fluid through the kidney and when these are structurally deformed or immotile, fluid egress is reduced and cysts develop in the anterior-most pronephric tubules. |
In this animation, cross sections of wild-type and mutant pronephroi are compared. The mutants double bubble (dbb) and fleer (flr) show distension of the glomerulus at the midline and flattening of the epithelium of the pronephric tubule as it becomes involved in the expanding cysts. |
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