WT1-MEDIATED GROWTH SUPPRESSION OF WILMS-TUMOR CELLS EXPRESSING A WT1 SPLICING VARIANT

被引：169

作者：

HABER, DA

PARK, S

MAHESWARAN, S

ENGLERT, C

RE, GG

HAZENMARTIN, DJ

SENS, DA

GARVIN, AJ

机构：

[1] HARVARD UNIV,SCH MED,BOSTON,MA 02129

[2] MED UNIV S CAROLINA,DEPT PATHOL & LAB MED,CHARLESTON,SC 29425

来源：

SCIENCE | 1993年 / 262卷 / 5142期

关键词：

D O I：

10.1126/science.8266105

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

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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页码：2057 / 2059

页数：3

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