Stewart Fleming
Murine Denys-Drash syndrome: Evidence of podocyte de-differentiation and systemic mediation of glomerulosclerosis
Fleming, Stewart; Hastie, Nicholas D.; Patek, Charles E.; Miles, Colin G.; Bellamy, Christopher O.; Ladomery, Michael; Spraggon, Lee; Mullins, John; Hooper, Martin L.
Authors
Nicholas D. Hastie
Charles E. Patek
Colin G. Miles
Christopher O. Bellamy
Prof Michael Ladomery Michael.Ladomery@uwe.ac.uk
Professor of Genetics
Lee Spraggon
John Mullins
Martin L. Hooper
Abstract
Denys-Drash syndrome (DDS) is caused by dominant mutations of the Wilms' tumour suppressor gene, WT1, and characterized by a nephropathy involving diffuse mesangial sclerosis, male pseudohermaphroditism and/ or Wilms' tumourigenesis. Previously, we reported that heterozygosity for the Wt1tmT396 mutation induces DDS in heterozygous and chimeric (Wt1tmT396/+ ↔ +/+) mice. In the present study, the fate of Wt1 mutant cells in chimeric kidneys was assessed by in situ marker analysis, and immunocytochemistry was used to re-examine the claim that glomerulosclerosis (GS) is caused by loss of WT1 and persistent Pax-2 expression by podocytes. Wt1 mutant cells colonized glomeruli efficiently, including podocytes, but some sclerotic glomeruli contained no detectable Wt1 mutant cells. The development of GS was preceded by widespread loss of ZO-1 signal in podocytes (even in kidneys where
Journal Article Type | Review |
---|---|
Publication Date | Sep 15, 2003 |
Journal | Human Molecular Genetics |
Print ISSN | 0964-6906 |
Publisher | Oxford University Press (OUP) |
Peer Reviewed | Peer Reviewed |
Volume | 12 |
Issue | 18 |
Pages | 2379-2394 |
DOI | https://doi.org/10.1093/hmg/ddg240 |
Public URL | https://uwe-repository.worktribe.com/output/1074282 |
Publisher URL | http://dx.doi.org/10.1093/hmg/ddg240 |
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