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PMID:16672320
| Citation |
Dziarmaga, A, Eccles, M and Goodyer, P (2006) Suppression of ureteric bud apoptosis rescues nephron endowment and adult renal function in Pax2 mutant mice. J. Am. Soc. Nephrol. 17:1568-75 |
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| Abstract |
The molecular mechanisms that set congenital nephron number are unknown. However, humans with modest suboptimal nephron number may be at increased risk for essential hypertension, and those with more severe nephron deficits at birth may develop progressive renal insufficiency. A model of branching morphogenesis during fetal kidney development in which the extent of ureteric bud arborization is dependent on suppression of programmed cell death has been proposed. This study shows that the increased apoptosis and reduced ureteric bud branching of heterozygous Pax2 mutant mice is associated with 40% decrease in nephron number at birth. This leads to postnatal glomerular hypertrophy and long-term renal insufficiency in the absence of glomerulosclerosis. To determine whether restoration of antiapoptotic factors alone is sufficient to rescue the nephron deficit in these mice, a BCL2 transgene that is under the control of the PAX2 promoter was targeted to the ureteric bud. The transgene suppressed programmed cell death in the ureteric bud lineage, increased nephron number to 90% of that of wild-type littermates at birth, and normalized renal function at 1 yr. These observations lend strong support to the hypothesis that factors that control ureteric bud apoptosis are powerful determinants of congenital nephron endowment. |
| Links |
PubMed Online version:10.1681/ASN.2005101074 |
| Keywords |
Animals; Apoptosis; Green Fluorescent Proteins/metabolism; Heterozygote; Kidney/embryology; Kidney/pathology; Kidney Glomerulus/pathology; Mice; Mice, Mutant Strains; Mice, Transgenic; Mutation; Nephrons/pathology; PAX2 Transcription Factor/genetics; PAX2 Transcription Factor/physiology; Transgenes; Ureter/embryology; Ureter/pathology |
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Significance
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| Gene product | Qualifier | GO ID | GO term name | Evidence Code | with/from | Aspect | Notes | Status |
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