Article
An inducible mouse model of podocin-mutation-related nephrotic syndrome.
PloS one - 1 Jan 2017
Tabatabaeifar Mansoureh, Wlodkowski Tanja, Simic Ivana, Denc Helga, Mollet Geraldine, Weber Stefanie, Moyers John Julius, Brühl Barbara, Randles Michael Joseph, Lennon Rachel, Antignac Corinne, Schaefer Franz
Abstract excerpt
Mutations in the NPHS2 gene, encoding podocin, cause hereditary nephrotic syndrome. The most common podocin mutation, R138Q, is associated with early disease onset and rapid progression to end-stage renal disease. Knock-in mice carrying a R140Q mutation, the mouse analogue of human R138Q, show developmental arrest of podocytes and lethal renal failure at neonatal age. Here we created a conditional podocin...
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