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Mutations in Bcl9 and Pygo genes cause congenital heart defects by tissue-specific perturbation of Wnt/β-catenin signaling
Bcl9 and Pygopus (Pygo) are obligate Wnt/β-catenin cofactors in Drosophila, yet their contribution to Wnt signaling during vertebrate development remains unresolved. Combining zebrafish and mouse genetics, we document a conserved, β-catenin-associated function for BCL9 and Pygo proteins during verte...
| Autores principales: | , , , , , , , , , , , , , , , , , |
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| Formato: | Online Artículo Texto |
| Lenguaje: | English |
| Publicado: |
Cold Spring Harbor Laboratory Press
2018
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| Materias: | |
| Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6217730/ https://www.ncbi.nlm.nih.gov/pubmed/30366904 http://dx.doi.org/10.1101/gad.315531.118 |
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| author | Cantù, Claudio Felker, Anastasia Zimmerli, Dario Prummel, Karin D. Cabello, Elena M. Chiavacci, Elena Méndez-Acevedo, Kevin M. Kirchgeorg, Lucia Burger, Sibylle Ripoll, Jorge Valenta, Tomas Hausmann, George Vilain, Nathalie Aguet, Michel Burger, Alexa Panáková, Daniela Basler, Konrad Mosimann, Christian |
| author_facet | Cantù, Claudio Felker, Anastasia Zimmerli, Dario Prummel, Karin D. Cabello, Elena M. Chiavacci, Elena Méndez-Acevedo, Kevin M. Kirchgeorg, Lucia Burger, Sibylle Ripoll, Jorge Valenta, Tomas Hausmann, George Vilain, Nathalie Aguet, Michel Burger, Alexa Panáková, Daniela Basler, Konrad Mosimann, Christian |
| author_sort | Cantù, Claudio |
| collection | PubMed |
| description | Bcl9 and Pygopus (Pygo) are obligate Wnt/β-catenin cofactors in Drosophila, yet their contribution to Wnt signaling during vertebrate development remains unresolved. Combining zebrafish and mouse genetics, we document a conserved, β-catenin-associated function for BCL9 and Pygo proteins during vertebrate heart development. Disrupting the β-catenin–BCL9–Pygo complex results in a broadly maintained canonical Wnt response yet perturbs heart development and proper expression of key cardiac regulators. Our work highlights BCL9 and Pygo as selective β-catenin cofactors in a subset of canonical Wnt responses during vertebrate development. Moreover, our results implicate alterations in BCL9 and BCL9L in human congenital heart defects. |
| format | Online Article Text |
| id | pubmed-6217730 |
| institution | National Center for Biotechnology Information |
| language | English |
| publishDate | 2018 |
| publisher | Cold Spring Harbor Laboratory Press |
| record_format | MEDLINE/PubMed |
| spelling | pubmed-62177302019-05-01 Mutations in Bcl9 and Pygo genes cause congenital heart defects by tissue-specific perturbation of Wnt/β-catenin signaling Cantù, Claudio Felker, Anastasia Zimmerli, Dario Prummel, Karin D. Cabello, Elena M. Chiavacci, Elena Méndez-Acevedo, Kevin M. Kirchgeorg, Lucia Burger, Sibylle Ripoll, Jorge Valenta, Tomas Hausmann, George Vilain, Nathalie Aguet, Michel Burger, Alexa Panáková, Daniela Basler, Konrad Mosimann, Christian Genes Dev Research Paper Bcl9 and Pygopus (Pygo) are obligate Wnt/β-catenin cofactors in Drosophila, yet their contribution to Wnt signaling during vertebrate development remains unresolved. Combining zebrafish and mouse genetics, we document a conserved, β-catenin-associated function for BCL9 and Pygo proteins during vertebrate heart development. Disrupting the β-catenin–BCL9–Pygo complex results in a broadly maintained canonical Wnt response yet perturbs heart development and proper expression of key cardiac regulators. Our work highlights BCL9 and Pygo as selective β-catenin cofactors in a subset of canonical Wnt responses during vertebrate development. Moreover, our results implicate alterations in BCL9 and BCL9L in human congenital heart defects. Cold Spring Harbor Laboratory Press 2018-11-01 /pmc/articles/PMC6217730/ /pubmed/30366904 http://dx.doi.org/10.1101/gad.315531.118 Text en © 2018 Cantù et al.; Published by Cold Spring Harbor Laboratory Press http://creativecommons.org/licenses/by-nc/4.0/ This article is distributed exclusively by Cold Spring Harbor Laboratory Press for the first six months after the full-issue publication date (see http://genesdev.cshlp.org/site/misc/terms.xhtml). After six months, it is available under a Creative Commons License (Attribution-NonCommercial 4.0 International), as described at http://creativecommons.org/licenses/by-nc/4.0/. |
| spellingShingle | Research Paper Cantù, Claudio Felker, Anastasia Zimmerli, Dario Prummel, Karin D. Cabello, Elena M. Chiavacci, Elena Méndez-Acevedo, Kevin M. Kirchgeorg, Lucia Burger, Sibylle Ripoll, Jorge Valenta, Tomas Hausmann, George Vilain, Nathalie Aguet, Michel Burger, Alexa Panáková, Daniela Basler, Konrad Mosimann, Christian Mutations in Bcl9 and Pygo genes cause congenital heart defects by tissue-specific perturbation of Wnt/β-catenin signaling |
| title | Mutations in Bcl9 and Pygo genes cause congenital heart defects by tissue-specific perturbation of Wnt/β-catenin signaling |
| title_full | Mutations in Bcl9 and Pygo genes cause congenital heart defects by tissue-specific perturbation of Wnt/β-catenin signaling |
| title_fullStr | Mutations in Bcl9 and Pygo genes cause congenital heart defects by tissue-specific perturbation of Wnt/β-catenin signaling |
| title_full_unstemmed | Mutations in Bcl9 and Pygo genes cause congenital heart defects by tissue-specific perturbation of Wnt/β-catenin signaling |
| title_short | Mutations in Bcl9 and Pygo genes cause congenital heart defects by tissue-specific perturbation of Wnt/β-catenin signaling |
| title_sort | mutations in bcl9 and pygo genes cause congenital heart defects by tissue-specific perturbation of wnt/β-catenin signaling |
| topic | Research Paper |
| url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6217730/ https://www.ncbi.nlm.nih.gov/pubmed/30366904 http://dx.doi.org/10.1101/gad.315531.118 |
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