XB-ART-12425
Proc Natl Acad Sci U S A
1999 Aug 31;9618:10212-7. doi: 10.1073/pnas.96.18.10212.
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Transcription repression by Xenopus ET and its human ortholog TBX3, a gene involved in ulnar-mammary syndrome.
He Ml
,
Wen L
,
Campbell CE
,
Wu JY
,
Rao Y
.
???displayArticle.abstract???
T box (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.
???displayArticle.pubmedLink??? 10468588
???displayArticle.pmcLink??? PMC17868
???displayArticle.link??? Proc Natl Acad Sci U S A
???displayArticle.grants??? [+]
DK48796 NIDDK NIH HHS , R01 CA114197-01A2 NCI NIH HHS , R01 EY014576-03 NEI NIH HHS , R01 GM070967-02 NIGMS NIH HHS , R01 CA114197 NCI NIH HHS , R01 EY014576 NEI NIH HHS , R01 GM070967 NIGMS NIH HHS , R01 DK048796 NIDDK NIH HHS
Species referenced: Xenopus laevis
Genes referenced: tbx2 tbx3
GO keywords: negative regulation of transcription, DNA-templated
???displayArticle.disOnts??? Holt-Oram syndrome
???displayArticle.omims??? HOLT-ORAM SYNDROME; HOS
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