Tbx1 regulates the BMP-Smad1 pathway in a transcription independent manner (Articolo in rivista)

Type
Label
  • Tbx1 regulates the BMP-Smad1 pathway in a transcription independent manner (Articolo in rivista) (literal)
Anno
  • 2009-01-01T00:00:00+01:00 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#doi
  • 10.1371/journal.pone.0006049 (literal)
Alternative label
  • Fulcoli G(1,5); Huynh T(2); Scambler PJ(3); Baldini A(1,2,4,5) (2009)
    Tbx1 regulates the BMP-Smad1 pathway in a transcription independent manner
    in PloS one
    (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
  • Fulcoli G(1,5); Huynh T(2); Scambler PJ(3); Baldini A(1,2,4,5) (literal)
Pagina inizio
  • e6049 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume
  • 4 (literal)
Rivista
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#note
  • PMID: 19557177 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#pagineTotali
  • 8 (literal)
Note
  • ISI Web of Science (WOS) (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
  • 1 Telethon Institute of Genetics and Medicine, Naples, Italy 2 Institute of Biosciences and Technology, Texas A&M University Health Sciences Center, Houston, Texas, United States of America 3 Institute of Child Health, London, United Kingdom 4 University Federico II, Naples, Italy 5 Institute of Genetics and Biophysics, CNR, Naples, Italy (literal)
Titolo
  • Tbx1 regulates the BMP-Smad1 pathway in a transcription independent manner (literal)
Abstract
  • Tbx1 is a T-box transcription factor implicated in DiGeorge syndrome. The molecular function of Tbx1 is unclear although it can transactivate reporters with T-box binding elements. We discovered that Tbx1 binds Smad1 and suppresses the Bmp4/ Smad1 signaling. Tbx1 interferes with Smad1 to Smad4 binding, and a mutation of Tbx1 that abolishes transactivation, does not affect Smad1 binding nor does affect the ability to suppress Smad1 activity. In addition, a disease-associated mutation of TBX1 that does not prevent transactivation, prevents the TBX1-SMAD1 interaction. Expression of Tbx1 in transgenic mice generates phenotypes similar to those associated with loss of a Bmp receptor. One phenotype could be rescued by transgenic Smad1 expression. Our data indicate that Tbx1 interferes with Bmp/Smad1 signaling and provide strong evidence that a T-box transcription factor has functions unrelated to transactivation. (literal)
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