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A critical developmental switch defines the kinetics of kidney cyst formation after loss of Pkd1.

Abstract：

:Autosomal dominant polycystic kidney disease is an important cause of end-stage renal disease, for which there is no proven therapy. Mutations in PKD1 (the gene encoding polycystin-1) are the principal cause of this disease. The disease begins in utero and is slowly progressive, but it is not known whether cystogenesis is an ongoing process during adult life. We now show that inactivation of Pkd1 in mice before postnatal day 13 results in severely cystic kidneys within 3 weeks, whereas inactivation at day 14 and later results in cysts only after 5 months. We found that cellular proliferation was not appreciably higher in cystic specimens than in age-matched controls, but the abrupt change in response to Pkd1 inactivation corresponded to a previously unrecognized brake point during renal growth and significant changes in gene expression. These findings suggest that the effects of Pkd1 inactivation are defined by a developmental switch that signals the end of the terminal renal maturation process. Our studies show that Pkd1 regulates tubular morphology in both developing and adult kidney, but the pathologic consequences of inactivation are defined by the organ's developmental status. These results have important implications for clinical understanding of the disease and therapeutic approaches.

journal_name

Nat Med

journal_title

Nature medicine

authors

Piontek K,Menezes LF,Garcia-Gonzalez MA,Huso DL,Germino GG

doi

10.1038/nm1675

subject

Has Abstract

pub_date

2007-12-01 00:00:00

pages

1490-5

issue

12

eissn

1078-8956

issn

1546-170X

pii

nm1675

journal_volume

13

pub_type

杂志文章

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