Polycystic kidney disease (PKD) causes internal epithelial cysts and afflicts patients with hernias, aneurysms and cardiac valve defects. Mangos et al. describe a zebrafish model of autosomal dominant PKD, in which the fish are deficient in the polycystin genes, pkd1a/b and pkd2, linked to human PKD. The main structural element, the tail, is disrupted in mutant fish owing to over-abundant collagen deposition. This suggests that polycystins influence tissue formation and demonstrates a regulatory role for the extracellular matrix in PKD.
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IN THIS ISSUE| 28 April 2010
Polycystins regulate tissue formation
Online Issn: 1754-8411
Print Issn: 1754-8403
Dis Model Mech (2010) 3 (5-6): 255.
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Polycystins regulate tissue formation. Dis Model Mech 28 April 2010; 3 (5-6): 255. doi:
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