@article{87b17dea8c4944bab6292cc119bf08ac,
title = "Role of Epiprofin, a zinc-finger transcription factor, in limb development",
abstract = "The formation and maintenance of the apical ectodermal ridge (AER) is critical for the outgrowth and patterning of the vertebrate limb. In the present work, we have investigated the role of Epiprofin (Epfn/Sp6), a member of the SP/KLF transcription factor family that is expressed in the limb ectoderm and the AER, during limb development. Epfn mutant mice have a defective autopod that shows mesoaxial syndactyly in the forelimb and synostosis (bony fusion) in the hindlimb and partial bidorsal digital tips. Epfn mutants also show a defect in the maturation of the AER that appears flat and broad, with a double ridge phenotype. By genetic analysis, we also show that Epfn is controlled by WNT/b-CATENIN signaling in the limb ectoderm. Since the less severe phenotypes of the conditional removal of b-catenin in the limb ectoderm strongly resemble the limb phenotype of Epfn mutants, we propose that EPFN very likely functions as a modulator of WNT signaling in the limb ectoderm.",
keywords = "AER, Apical ectodermal ridge, BMP, Epiprofin, Limb development, Oligodactyly, Sp/KLF, Sp6, Syndactyly, WNT/b-CATENIN",
author = "Ana Talamillo and Irene Delgado and Takashi Nakamura and Susana de-Vega and Yasuo Yoshitomi and Fernando Unda and Walter Birchmeier and Yoshihiko Yamada and Ros, {Maria A.}",
note = "Funding Information: We are very grateful to G. Martin and F. Mariani for the double Fgf4;Fgf8 mutant embryos and to J. Galceran, A. Joyner, G. Martin, A. McMahon and C. Niehrs for generously providing reagents. This work was supported by grant BFU2008-00397 from the Spanish Ministry of Education and Science and grant API 05/27 from the IFIMAV to M.R., the Intramural Research Program of the NIDCR, NIH to Y.Y. and grant GIU05/27 from the University of the Basque Country to F.U. A.T. had a postdoctoral fellowship from the Instituto de Formaci{\'o}n e Investigaci{\'o}n Marqu{\'e}s de Valdecilla (IFIMAV). We acknowledge the excellent technical assistance of Marisa Junco. ",
year = "2010",
month = jan,
day = "15",
doi = "10.1016/j.ydbio.2009.11.007",
language = "English",
volume = "337",
pages = "363--374",
journal = "Developmental Biology",
issn = "0012-1606",
publisher = "Academic Press Inc.",
number = "2",
}