Cited by 120Developmental Biology and Gene RegulationGenomics and Chromatin DynamicsCongenital limb and hand anomalies

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

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He, Ming-liang, Wen, Leng, Campbell, Christine E., Wu, Jane Y., Rao, Yi·1999·Proceedings of the National Academy of Sciences

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.

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Authors
He, M.-L., Wen, L., Campbell, C. E., Wu, J. Y., & Rao, Y.
Affiliations
Cleveland Clinic (US)
Venue
Proceedings of the National Academy of Sciences
Published
1999-08-31
Volume
96
Issue
18
Pages
10212-10217
ISSN
  • 0027-8424 (ISSN-L)
  • 1091-6490
OpenAlex
W2018078167
Cited by
120
References
98
Citations by year
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FWCI
3.10
Type
journal article
Language
en
Open access
Locations
  • Proceedings of the National Academy of Sciences (publishedVersion)
  • Europe PMC (PubMed Central) (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • National Science Foundation (9723173)
  • John Merck Fund
  • National Institutes of Health
  • National Eye Institute (R01EY014576)
  • National Cancer Institute (R01CA114197)
  • National Institute of General Medical Sciences (R01GM070967)
  • National Institute of Diabetes and Digestive and Kidney Diseases (R01DK048796, DK48796)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Developmental Biology and Gene Regulation
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Genomics and Chromatin Dynamics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Developmental Biology › Congenital limb and hand anomalies
Keywords
TBX3, TBX2, T-box genes, transcriptional repression, ulnar-mammary syndrome, Xenopus embryo, Brachyury, transcriptional regulation
MeSH terms
Congenital Abnormalities, Amino Acid Sequence, Animals, Breast, Cell Line, Embryo, Nonmammalian, Humans, Molecular Sequence Data, Recombinant Fusion Proteins, Repressor Proteins, Syndrome, Transcription Factors, Transcription, Genetic, Ulna, Xenopus laevis, Sequence Alignment, Sequence Homology, Amino Acid, Gene Expression Regulation, Developmental, T-Box Domain Proteins, Xenopus Proteins

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