PMID- 10468588 OWN - NLM STAT- MEDLINE DCOM- 19991007 LR - 20210109 IS - 0027-8424 (Print) IS - 0027-8424 (Linking) VI - 96 IP - 18 DP - 1999 Aug 31 TI - Transcription repression by Xenopus ET and its human ortholog TBX3, a gene involved in ulnar-mammary syndrome. PG - 10212-7 AB - 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. FAU - He, M l AU - He Ml AD - Department of Anatomy and Neurobiology, Washington University School of Medicine, St. Louis, MO 63110, USA. FAU - Wen, L AU - Wen L FAU - Campbell, C E AU - Campbell CE FAU - Wu, J Y AU - Wu JY FAU - Rao, Y AU - Rao Y LA - eng SI - GENBANK/AF170708 SI - GENBANK/AF173940 GR - R01 EY014576-03/EY/NEI NIH HHS/United States GR - DK48796/DK/NIDDK NIH HHS/United States GR - R01 GM070967-02/GM/NIGMS NIH HHS/United States GR - R01 DK048796/DK/NIDDK NIH HHS/United States GR - R01 CA114197-01A2/CA/NCI NIH HHS/United States GR - R01 EY014576/EY/NEI NIH HHS/United States GR - R01 GM070967/GM/NIGMS NIH HHS/United States GR - R01 CA114197/CA/NCI NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, U.S. Gov't, Non-P.H.S. PT - Research Support, U.S. Gov't, P.H.S. PL - United States TA - Proc Natl Acad Sci U S A JT - Proceedings of the National Academy of Sciences of the United States of America JID - 7505876 RN - 0 (Recombinant Fusion Proteins) RN - 0 (Repressor Proteins) RN - 0 (T-Box Domain Proteins) RN - 0 (TBX3 protein, human) RN - 0 (Tbx3 protein, Xenopus) RN - 0 (Transcription Factors) RN - 0 (Xenopus Proteins) RN - 0 (transcription repression factor ET, Xenopus) SB - IM EIN - Proc Natl Acad Sci U S A 1999 Nov 9;96(23):13589 MH - Amino Acid Sequence MH - Animals MH - Breast/*abnormalities MH - Cell Line MH - Congenital Abnormalities/*genetics MH - Embryo, Nonmammalian/metabolism MH - Gene Expression Regulation, Developmental MH - Humans MH - Molecular Sequence Data MH - Recombinant Fusion Proteins/biosynthesis MH - Repressor Proteins/chemistry/*genetics MH - Sequence Alignment MH - Sequence Homology, Amino Acid MH - Syndrome MH - *T-Box Domain Proteins MH - Transcription Factors/chemistry/*genetics MH - *Transcription, Genetic MH - Ulna/*abnormalities MH - *Xenopus Proteins MH - Xenopus laevis/embryology/genetics PMC - PMC17868 EDAT- 1999/09/01 09:00 MHDA- 2000/03/04 09:00 CRDT- 1999/09/01 09:00 PHST- 1999/09/01 09:00 [pubmed] PHST- 2000/03/04 09:00 [medline] PHST- 1999/09/01 09:00 [entrez] AID - 10.1073/pnas.96.18.10212 [doi] PST - ppublish SO - Proc Natl Acad Sci U S A. 1999 Aug 31;96(18):10212-7. doi: 10.1073/pnas.96.18.10212.