WT1-mediated growth suppression of Wilms tumor cells expressing a WT1 splicing variant - PubMed (original) (raw)
. 1993 Dec 24;262(5142):2057-9.
doi: 10.1126/science.8266105.
Affiliations
- PMID: 8266105
- DOI: 10.1126/science.8266105
WT1-mediated growth suppression of Wilms tumor cells expressing a WT1 splicing variant
D A Haber et al. Science. 1993.
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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