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

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

[Expression of WT1 gene mRNA in leukemia]
The Wilms tumor suppressor WT1 regulates early gonad development by activation of Sf1.
A review of the phenotypic variation due to the Denys-Drash syndrome-associated germline WT1 mutation R362X.

Table of Contents

1
UI - 12080641
AU - Jian Z; Chen F; Xie Q; Liu W; Zhong M
TI - [Expression of WT1 gene mRNA in leukemia]
SO - Hunan Yi Ke Da Xue Xue Bao 1999;24(4):341-2
AD - Department of Hematology, Xiangya Hospital, Hunan Medical University, Changsha 410008.
WT1 gene expression was determined in acute leukemic patients by RT-PCR. RESULTS: 19 patients were found to have positive results among 28 patients, the positive rate is 67.9%, and in 16 ANLL patients, 11 cases got positive results, in 12 ALL patients, 8 cases got positive results. It suggested that WT1 was highly expressed in acute leukemia. It is a new and significant detective marker for monitoring the effect of chemotherapy, predicting the prognosis and in the research of minimal residual disease (MRD).

2
UI - 12130543
AU - Wilhelm D; Englert C
TI - The Wilms tumor suppressor WT1 regulates early gonad development by activation of Sf1.
SO - Genes Dev 2002 Jul 15;16(14):1839-51
AD - Institut fur Toxikologie und Genetik, Forschungszentrum Karlsruhe, 76021 Karlsruhe, Germany.
In mammals, several genes including the Wilms tumor suppressor gene Wt1, the Lim homeobox gene Lhx9, and the gene encoding steroidogenic factor 1 (Sf1) have been implicated in the development of the indifferent gonad prior to sexual differentiation. Interactions among these genes have not yet been elucidated. Using biochemical and genetic experiments, we demonstrate here that WT1 and LHX9 function as direct activators of the Sf1 gene. Interestingly, only the -KTS form of WT1 is able to bind to and transactivate the Sf1 promoter. This observation is consistent with differential roles for the -KTS and +KTS variants of WT1 which have been postulated on the basis of human disorders such as the Frasier syndrome. Our data suggest a pathway in which the products of the Wt1 and Lhx9 genes activate expression of Sf1 and thus mediate early gonadogenesis.

3
UI - 11933209
AU - Heathcott RW; Morison IM; Gubler MC; Corbett R; Reeve AE
TI - A review of the phenotypic variation due to the Denys-Drash syndrome-associated germline WT1 mutation R362X.
SO - Hum Mutat 2002 Apr;19(4):462
AD - Cancer Genetics Laboratory, Department of Biochemistry, University of Otago, Dunedin, New Zealand.
The gene WT1 is required for the normal development and function of the urogenital tract. Constitutional mutations are associated with familial Wilms tumor and syndromes such as Denys-Drash syndrome (DDS) characterized by nephropathy, genital anomalies and often a predisposition to Wilms tumor. We report a case of constitutional WT1 mutation in an XX female with multifocal Wilms tumor but no genital anomalies or renal dysfunction and, for the first time, review patients previously reported with this germline mutation. The mutation (1084C>T) changes the amino acid arginine at position 362 to the translation stop codon TGA (R362X) resulting in a predicted truncated protein lacking three of the four zinc finger domains necessary for correct functioning of the gene. This constitutional mutation has been reported to cause a variety of phenotypes in eleven different patients, including the classical Denys-Drash phenotype of diffuse mesangial sclerosis which leads to early renal failure, genital anomalies in XY individuals and Wilms tumors. The absence of mesangial sclerosis and renal failure in our patient excludes DDS. Our case differs from those previously described as the normal kidney tissue shows some small subcapsular glomeruli indicating that the WT1 mutation has impaired nephron development. This patient extends the range and variation of phenotypes that may arise from a specific germline mutation in WT1. Copyright 2002 Wiley-Liss, Inc.

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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