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NCI CANCERLIT^® Search: Wilms' Tumor Gene (WT1) - October 2002

National Cancer Institute^®
Last Modified: October 1, 2002

A girl with bilateral ovarian tumours: Frasier syndrome.
Expression of a gene encoding a novel potential IGF binding protein in human tissues.

Table of Contents

1
UI - 11954756
AU - Shimoyama H; Nakajima M; Naka H; Park YD; Hori K; Morikawa H; Yoshioka A
TI - A girl with bilateral ovarian tumours: Frasier syndrome.
SO - Eur J Pediatr 2002 Feb;161(2):81-3
AD - Department of Paediatrics, Nara Medical University, Kashihara City, Japan. hironobu@nmu-gw.naramed-u.ac.jp
Frasier syndrome (FS) is characterised by male pseudohermaphroditism, slowly progressing nephropathy and frequent development of gonadoblastoma. The Wilms' tumour suppressor gene (WT1 gene) plays an important role in the development of the urogenital system and the gonads. A splice mutation in intron 9 of the WT1 gene was recently described in patients with FS. We analysed the WT1 gene of a Japanese patient with male pseudohermaphroditism, steroid resistant-nephr-opathy and gonadoblastoma by the polymerase chain reaction and direct sequencing and detected a heterozygous point mutation in intron 9. CONCLUSION: analysis of the Wilms' tumour suppressor gene in a patient with Frasier syndrome by the polymerase chain reaction and direct sequencing detected a + 5G -->A transition at a position of the second alternative splice region of exon 9, important for predicting the risk of the occurrence of Wilms' tumour.

2
UI - 1756408
AU - Martinerie C; Perbal B
TI - Expression of a gene encoding a novel potential IGF binding protein in human tissues.
SO - C R Acad Sci III 1991;313(8):345-51
AD - Laboratoire d'Oncologie virale et moleculaire, Institut Curie, Centre Universitaire, Orsay.
We have shown in a previous study that the expression of an as yet unidentified, embryonic gene (nov) encoding a potential IGF binding protein was upregulated in all of eight virally-induced avian nephroblastomas tested. We now report that homologous sequences are conserved in human DNA and are expressed in normal human bone marrow, thymic cells and in one nephroblastoma.

The above citations and abstracts reflect those newly added to CANCERLIT for the month and topic listed in the title. The citations have been retrieved from CANCERLIT using a predefined search strategy of indexed subject terms. Although the search strategy has been refined as best as possible, citations may appear that are not directly related to the topic, and occasionally relevant references may be omitted.

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