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. 1993 Dec 24;262(5142):2057-9.
doi: 10.1126/science.8266105.

WT1-mediated growth suppression of Wilms tumor cells expressing a WT1 splicing variant

D A Haber  1 , S Park, S Maheswaran, C Englert, G G Re, D J Hazen-Martin, D A Sens, A J Garvin
Affiliations

WT1-mediated growth suppression of Wilms tumor cells expressing a WT1 splicing variant

D A Haber et al. Science. .

Abstract

A human Wilms tumor cell line (RM1) was developed to test the tumor suppressor activity of WT1, a zinc finger transcription factor that is expressed in the developing human kidney and is mutationally inactivated in a subset of Wilms tumors. Transfection of each of four wild-type WT1 isoforms suppressed the growth of RM1 cells. The endogenous WT1 transcript in these cells was devoid of exon 2 sequences, a splicing alteration that was also detected in varying amounts in all Wilms tumors tested but not in normal kidney. Production of this abnormal transcript, which encodes a functionally altered protein, may represent a distinct mechanism for inactivating WT1 in Wilms tumors.

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