WT1 -Mediated Growth Suppression of Wilms Tumor Cells Expressing a WT1 Splicing Variant
- 24 December 1993
- journal article
- other
- Published by American Association for the Advancement of Science (AAAS) in Science
- Vol. 262 (5142), 2057-2059
- https://doi.org/10.1126/science.8266105
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.Keywords
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