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Publication : Disruption of transforming growth factor-beta signaling in ELF beta-spectrin-deficient mice.

First Author  Tang Y Year  2003
Journal  Science Volume  299
Issue  5606 Pages  574-7
PubMed ID  12543979 Mgi Jnum  J:81747
Mgi Id  MGI:2449899 Doi  10.1126/science.1075994
Citation  Tang Y, et al. (2003) Disruption of transforming growth factor-beta signaling in ELF beta-spectrin-deficient mice. Science 299(5606):574-7
abstractText  Disruption of the adaptor protein ELF, a beta-spectrin, leads to disruption of transforming growth factor-beta (TGF-beta) signaling by Smad proteins in mice. Elf-/- mice exhibit a phenotype similar to smad2+/-/smad3+/- mutant mice of midgestational death due to gastrointestinal, liver, neural, and heart defects. We show that TGF-beta triggers phosphorylation and association of ELF with Smad3 and Smad4, followed by nuclear translocation. ELF deficiency results in mislocalization of Smad3 and Smad4 and loss of the TGF-beta-dependent transcriptional response, which could be rescued by overexpression of the COOH-terminal region of ELF. This study reveals an unexpected molecular link between a major dynamic scaffolding protein and a key signaling pathway.
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