Megabladder mouse model of congenital obstructive nephropathy: genetic etiology and renal adaptation

Congenital obstructive nephropathy remains one of the leading causes of chronic renal failure in children. The direct link between obstructed urine flow and abnormal renal development and subsequent dysfunction represents a central paradigm of urogenital pathogenesis that has far-reaching clinical i...

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Bibliographic Details
Published in:Pediatric nephrology (Berlin, West) West), 2014-04, Vol.29 (4), p.645-650
Main Author: McHugh, Kirk M.
Format: Article
Language:English
Subjects:
Animals
Bladder
Chromosomes
Chronic kidney failure
Complications and side effects
Congenital diseases
Defects
Development and progression
Disease
Disease Models, Animal
Etiology
Genes
Genetic engineering
Genotype & phenotype
Hydronephrosis
Hydronephrosis - congenital
Hydronephrosis - pathology
Kidneys
Medicine
Medicine & Public Health
Mice
Nephrology
Pathogenesis
Pediatrics
Physiological aspects
Review
Risk factors
Smooth muscle
Urogenital system
Urologic diseases
Urology
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Summary:Congenital obstructive nephropathy remains one of the leading causes of chronic renal failure in children. The direct link between obstructed urine flow and abnormal renal development and subsequent dysfunction represents a central paradigm of urogenital pathogenesis that has far-reaching clinical implications. Even so, a number of diagnostic, prognostic, and therapeutic quandaries still exist in the management of congenital obstructive nephropathy. Studies in our laboratory have characterized a unique mutant mouse line that develops in utero megabladder, variable hydronephrosis, and progressive renal failure. Megabladder mice represent a valuable functional model for the study of congenital obstructive nephropathy. Recent studies have begun to shed light on the genetic etiology of mgb −/− mice as well as the molecular pathways controlling disease progression in these animals.
ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-013-2658-6