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Items: 1 to 20 of 109

1.

Effects on kidney disease, fertility and development in mice inheriting a protein-truncating Denys-Drash syndrome allele (Wt1tmT396).

Patek CE, Brownstein DG, Fleming S, Wroe C, Rose L, Webb A, Berry RL, Devenney PS, Walker M, Maddocks OD, Lawrence NJ, Harrison DJ, Wood KM, Miles CG, Hooper ML.

Transgenic Res. 2008 Jun;17(3):459-75. Epub 2007 Nov 27.

PMID:
18040647
2.

Murine Denys-Drash syndrome: evidence of podocyte de-differentiation and systemic mediation of glomerulosclerosis.

Patek CE, Fleming S, Miles CG, Bellamy CO, Ladomery M, Spraggon L, Mullins J, Hastie ND, Hooper ML.

Hum Mol Genet. 2003 Sep 15;12(18):2379-94. Epub 2003 Jul 22.

PMID:
12915483
3.

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

Patek CE, Little MH, Fleming S, Miles C, Charlieu JP, Clarke AR, Miyagawa K, Christie S, Doig J, Harrison DJ, Porteous DJ, Brookes AJ, Hooper ML, Hastie ND.

Proc Natl Acad Sci U S A. 1999 Mar 16;96(6):2931-6.

4.

A novel WT1 heterozygous nonsense mutation (p.K248X) causing a mild and slightly progressive nephropathy in a 46,XY patient with Denys-Drash syndrome.

da Silva TE, Nishi MY, Costa EM, Martin RM, Carvalho FM, Mendonca BB, Domenice S.

Pediatr Nephrol. 2011 Aug;26(8):1311-5. doi: 10.1007/s00467-011-1847-4. Epub 2011 May 11.

PMID:
21559934
5.

Effects of Denys-Drash syndrome point mutations on the DNA binding activity of the Wilms' tumor suppressor protein WT1.

Borel F, Barilla KC, Hamilton TB, Iskandar M, Romaniuk PJ.

Biochemistry. 1996 Sep 17;35(37):12070-6.

PMID:
8810912
6.

The Wt1+/R394W mouse displays glomerulosclerosis and early-onset renal failure characteristic of human Denys-Drash syndrome.

Gao F, Maiti S, Sun G, Ordonez NG, Udtha M, Deng JM, Behringer RR, Huff V.

Mol Cell Biol. 2004 Nov;24(22):9899-910.

7.

A novel WT1 gene mutation in a newborn infant diagnosed with Denys-Drash syndrome.

Hakan N, Aydin M, Erdogan O, Cavusoglu YH, Aycan Z, Ozaltin F, Zenciroglu A, Apaydin S, Gunes R, Sahin G, Cinar G, Okumus N.

Genet Couns. 2012;23(2):255-61.

PMID:
22876585
8.

Clinical pictures and novel mutations of WT1-associated Denys-Drash syndrome in two Chinese children.

Yue Z, Pei Y, Sun L, Huang W, Huang H, Hu B, Yang J, Jiang X, Mo Y, Chen S, Lai KN, Wang Y.

Ren Fail. 2011;33(9):910-4. doi: 10.3109/0886022X.2011.605528. Epub 2011 Aug 18.

PMID:
21851196
9.

A familial WT1 mutation associated with incomplete Denys-Drash syndrome.

Zhu C, Zhao F, Zhang W, Wu H, Chen Y, Ding G, Zhang A, Huang S.

Eur J Pediatr. 2013 Oct;172(10):1357-62. doi: 10.1007/s00431-013-2004-9. Epub 2013 May 29.

PMID:
23715653
10.

Gonadal effects of a mouse Denys-Drash syndrome mutation.

Patek CE, Saunders PT, Miles CG, Berry RL, Hastie ND, Sharpe RM, Hooper ML.

Transgenic Res. 2005 Oct;14(5):691-702.

PMID:
16245160
11.

A mutant form of the Wilms' tumor suppressor gene WT1 observed in Denys-Drash syndrome interferes with glomerular capillary development.

Natoli TA, Liu J, Eremina V, Hodgens K, Li C, Hamano Y, Mundel P, Kalluri R, Miner JH, Quaggin SE, Kreidberg JA.

J Am Soc Nephrol. 2002 Aug;13(8):2058-67.

12.

WT1 and PAX-2 podocyte expression in Denys-Drash syndrome and isolated diffuse mesangial sclerosis.

Yang Y, Jeanpierre C, Dressler GR, Lacoste M, Niaudet P, Gubler MC.

Am J Pathol. 1999 Jan;154(1):181-92.

13.

WT1 Haploinsufficiency Supports Milder Renal Manifestation in Two Patients with Denys-Drash Syndrome.

Guaragna MS, Ribeiro de Andrade JG, de Freitas Carli B, Belangero VM, Maciel-Guerra AT, Guerra-Júnior G, de Mello MP.

Sex Dev. 2017;11(1):34-39. doi: 10.1159/000454821. Epub 2017 Jan 13.

PMID:
28081536
14.

Hemolytic uremic syndrome as the presenting manifestation of WT1 mutation and Denys-Drash syndrome: a case report.

Alge JL, Wenderfer SE, Hicks J, Bekheirnia MR, Schady DA, Kain JS, Braun MC.

BMC Nephrol. 2017 Jul 18;18(1):243. doi: 10.1186/s12882-017-0643-1.

15.

A review of the phenotypic variation due to the Denys-Drash syndrome-associated germline WT1 mutation R362X.

Heathcott RW, Morison IM, Gubler MC, Corbett R, Reeve AE.

Hum Mutat. 2002 Apr;19(4):462. Review.

PMID:
11933209
16.

[Glomerulopathy in Denys-Drash syndrome. Case report of a model disease].

Stallmach T, Neuhaus TJ, Kösters R, Hailemariam S.

Pathologe. 1998 May;19(3):230-4. German.

PMID:
9648150
17.

[Denys-Drash syndrome. Experience gathered in Erlangen illustrated by two case reports].

Zugor V, Zenker M, Dötsch J, Schrott KM, Schott GE.

Urologe A. 2005 Oct;44(10):1197-200. German.

PMID:
16003530
18.

The novel WT1 gene mutation p.H377N associated to Denys-Drash syndrome.

Guaragna MS, Soardi FC, Assumpção JG, Zambaldi Lde J, Cardinalli IA, Yunes JA, de Mello MP, Brandalise SR, Aguiar Sdos S.

J Pediatr Hematol Oncol. 2010 Aug;32(6):486-8. doi: 10.1097/MPH.0b013e3181e5e20d.

PMID:
20562648
19.

WT1 and glomerular diseases.

Niaudet P, Gubler MC.

Pediatr Nephrol. 2006 Nov;21(11):1653-60. Epub 2006 Aug 23. Review.

PMID:
16927106
20.

[A genetic childhood disease with consequences in adult life: the Denys-Drash syndrome].

Löwik MM, van den Berkmortel FW, Noordam C, van Hamersvelt HW, van den Heuvel LP, Levtchenko EN.

Ned Tijdschr Geneeskd. 2005 Jul 30;149(31):1751-5. Dutch. Erratum in: Ned Tijdschr Geneeskd. 2005 Oct 1;149(40):2260.

PMID:
16114294

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