Transcription repression by Xenopus ET and its human ortholog TBX3, a gene involved in ulnar-mammary syndrome

Ming Liang He, Leng Wen, Christine E. Campbell, Jane Y. Wu, Yi Rao*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

104 Scopus citations

Abstract

Tbox (Tbx) genes are a family of developmental regulators with more than 20 members recently identified in invertebrates and vertebrates. Mutations in Tbx genes have been found to cause several human diseases. Our understanding of functional mechanisms of Tbx products has come mainly from the prototypical T/Brachyury, which is a transcription activator. We previously discovered ET, a Tbx gene expressed in Xenopus embryos. We report here that ET is an ortholog of the human Tbx3 and that ET is a repressor of basal and activated transcription. Functional dissection of the ET protein reveals a novel transcription-repression domain highly conserved among ET, human TBX3, and TBX2. These results reveal a new transcription repressor domain, show the existence of a subfamily of transcription repressors in the Tbx superfamily, and provide a basis for understanding etiology of diseases caused by Tbx3 mutations.

Original languageEnglish (US)
Pages (from-to)10212-10217
Number of pages6
JournalProceedings of the National Academy of Sciences of the United States of America
Volume96
Issue number18
DOIs
StatePublished - Aug 31 1999

ASJC Scopus subject areas

  • General

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