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Article

WT1 and glomerular diseases.

Pediatric nephrology (Berlin, Germany) - 1 Nov 2006

Niaudet Patrick, Gubler Marie-Claire

Abstract excerpt

The WT1 gene encodes a zinc finger transcription factor involved in kidney and gonadal development and, when mutated, in the occurrence of kidney tumor and glomerular diseases. Patients with Denys-Drash syndrome present with early nephrotic syndrome with diffuse mesangial sclerosis progressing rapidly to end-stage renal failure, male pseudohermaphroditism, and Wilms' tumor. Incomplete forms of the syndrome have...

Topics

Child
Chromosomes, Human, X
Chromosomes, Human, Y
Denys-Drash Syndrome
Female
Frasier Syndrome
Genes, Wilms Tumor
Genetic Counseling
Glomerular Mesangium
Humans
Kidney Diseases
Male

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Mutation

Nephrotic Syndrome

Sclerosis

Wilms Tumor

Source

Identifiers

PMID: 16927106
DOI: 10.1007/s00467-006-0208-1

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WT1 and glomerular diseases.PMID 16927106Read ↗

WT1 complete gonadal dysgenesis with membranoproliferative glomerulonephritis: case series and literature review.Semantic proximity Read ↗

WT1-related disorders: more than Denys-Drash syndrome.Semantic proximity Read ↗

Exploring the Multifaceted Role of WT1 in Kidney Development and DiseaseSemantic proximity Read ↗

Clinical spectrum of Denys-Drash and Frasier syndrome.Semantic proximity Read ↗

Two distinct WT1 mutations identified in patients and relatives with isolated nephrotic proteinuria.Semantic proximity Read ↗

A zinc finger truncation of murine WT1 results in the characteristic urogenital abnormalities of Denys-Drash syndrome.Semantic proximity Read ↗

Donor splice-site mutations in WT1 are responsible for Frasier syndrome.Semantic proximity Read ↗

Mother-to-child transmitted WT1 splice-site mutation is responsible for distinct glomerular diseases.Semantic proximity Read ↗

Clinical Aspects of WT1 and the Kidney.Semantic proximity Read ↗

Frasier syndrome, part of the Denys Drash continuum or simply a WT1 gene associated disorder of intersex and nephropathy?Semantic proximity Read ↗

Novel WT1 mutation (C388Y) in a female child with Denys-Drash syndrome.Semantic proximity Read ↗

An unusual phenotype of Frasier syndrome due to IVS9 +4C>T mutation in the WT1 gene: predominantly male ambiguous genitalia and absence of gonadal dysgenesis.Semantic proximity Read ↗

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