| Role of Epiprofin, a zinc-finger transcription factor, in limb development | | Role of Epiprofin, a zinc-finger transcription factor, in limb development | Talamillo A, Delgado I, Nakamura T, de-Vega S, Yoshitomi Y, Unda F, Birchmeier W, Yamada Y, Ros M. | 2010-01-14T23:00:00Z | <h2>Abstract</h2><p style="text-align:justify;"><span class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="box-sizing:border-box;margin:0px;padding:0px;color:#2e2e2e;"><span style="box-sizing:border-box;margin:0px;padding:0px;"><br></span></span></p><p style="text-align:justify;"><span class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="box-sizing:border-box;margin:0px;padding:0px;color:#2e2e2e;"><span style="box-sizing:border-box;margin:0px;padding:0px;">The formation and maintenance of the apical ectodermal ridge (AER) is critical for the outgrowth and patterning of the </span>vertebrate limb. In the present work, we have investigated the role of </span><em class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="box-sizing:border-box;margin:0px;padding:0px;color:#2e2e2e;">Epiprofin</em><span class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="color:#2e2e2e;"> (</span><em class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="box-sizing:border-box;margin:0px;padding:0px;color:#2e2e2e;">Epfn/Sp6</em><span class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="box-sizing:border-box;margin:0px;padding:0px;color:#2e2e2e;">), a member of the SP/KLF transcription factor family that is expressed in the limb ectoderm<span style="box-sizing:border-box;margin:0px;padding:0px;"> and the AER, during limb development. </span></span><em class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="box-sizing:border-box;margin:0px;padding:0px;color:#2e2e2e;">Epfn </em><span class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="box-sizing:border-box;margin:0px;padding:0px;color:#2e2e2e;">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. </span><em class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="box-sizing:border-box;margin:0px;padding:0px;color:#2e2e2e;">Epfn</em><span class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="color:#2e2e2e;"> 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 </span><em class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="box-sizing:border-box;margin:0px;padding:0px;color:#2e2e2e;">Epfn</em><span class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="color:#2e2e2e;"> is controlled by WNT/b-CATENIN signaling in the limb ectoderm. Since the less severe phenotypes of the conditional removal of </span><em class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="box-sizing:border-box;margin:0px;padding:0px;color:#2e2e2e;">b-catenin</em><span class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="color:#2e2e2e;"> in the limb ectoderm strongly resemble the limb phenotype of </span><em class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="box-sizing:border-box;margin:0px;padding:0px;color:#2e2e2e;">Epfn</em><span class="ms-rteThemeFontFace-1 ms-rteFontSize-2" style="color:#2e2e2e;">mutants, we propose that EPFN very likely functions as a modulator of WNT signaling in the limb ectoderm.</span><br></p> | <p><a href="https://www.sciencedirect.com/science/article/pii/S0012160609013451"><span class="ms-rteThemeFontFace-1 ms-rteFontSize-2 ms-rteThemeForeColor-5-0">Developmental Biology. 2010; 337:363.</span></a><strong></strong><span style="color:#000000;font-family:-webkit-standard;font-size:medium;"></span><br></p> | 214 | | |
