Eintrag weiter verarbeiten
Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer
Gespeichert in:
| Zeitschriftentitel: | Disease Models & Mechanisms |
|---|---|
| Personen und Körperschaften: | , , , , , , |
| In: | Disease Models & Mechanisms, 11, 2018, 9 |
| Format: | E-Article |
| Sprache: | Englisch |
| veröffentlicht: |
The Company of Biologists
|
| Schlagwörter: |
| author_facet |
Pane, Luna Simona Fulcoli, Filomena Gabriella Cirino, Andrea Altomonte, Alessandra Ferrentino, Rosa Bilio, Marchesa Baldini, Antonio Pane, Luna Simona Fulcoli, Filomena Gabriella Cirino, Andrea Altomonte, Alessandra Ferrentino, Rosa Bilio, Marchesa Baldini, Antonio |
|---|---|
| author |
Pane, Luna Simona Fulcoli, Filomena Gabriella Cirino, Andrea Altomonte, Alessandra Ferrentino, Rosa Bilio, Marchesa Baldini, Antonio |
| spellingShingle |
Pane, Luna Simona Fulcoli, Filomena Gabriella Cirino, Andrea Altomonte, Alessandra Ferrentino, Rosa Bilio, Marchesa Baldini, Antonio Disease Models & Mechanisms Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer General Biochemistry, Genetics and Molecular Biology Immunology and Microbiology (miscellaneous) Medicine (miscellaneous) Neuroscience (miscellaneous) |
| author_sort |
pane, luna simona |
| spelling |
Pane, Luna Simona Fulcoli, Filomena Gabriella Cirino, Andrea Altomonte, Alessandra Ferrentino, Rosa Bilio, Marchesa Baldini, Antonio 1754-8411 1754-8403 The Company of Biologists General Biochemistry, Genetics and Molecular Biology Immunology and Microbiology (miscellaneous) Medicine (miscellaneous) Neuroscience (miscellaneous) http://dx.doi.org/10.1242/dmm.029967 <jats:title>ABSTRACT</jats:title> <jats:p>The TBX1 gene is haploinsufficient in 22q11.2 deletion syndrome (22q11.2DS), and genetic evidence from human patients and mouse models points to a major role of this gene in the pathogenesis of this syndrome. Tbx1 can activate and repress transcription, and previous work has shown that one of its functions is to negatively modulate cardiomyocyte differentiation. Tbx1 occupies the anterior heart field (AHF) enhancer of the Mef2c gene, which encodes a key cardiac differentiation transcription factor. Here, we show that increased dosage of Tbx1 correlates with downregulation of Mef2c expression and reduced acetylation of its AHF enhancer in cultured mouse myoblasts. Consistently, 22q11.2DS-derived and in vitro-differentiated human induced pluripotent stem cells (hiPSCs) expressed higher levels of MEF2C and showed increased AHF acetylation, compared with hiPSCs from a healthy donor. Most importantly, we show that in mouse embryos, loss of Tbx1 enhances the expression of the Mef2c-AHF-Cre transgene in a specific region of the splanchnic mesoderm, and in a dosage-dependent manner, providing an in vivo correlate of our cell culture data. These results indicate that Tbx1 regulates the Mef2c AHF enhancer by inducing histone deacetylation.</jats:p> Tbx1 represses <i>Mef2c</i> gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer Disease Models & Mechanisms |
| doi_str_mv |
10.1242/dmm.029967 |
| facet_avail |
Online Free |
| finc_class_facet |
Medizin |
| format |
ElectronicArticle |
| fullrecord |
blob:ai-49-aHR0cDovL2R4LmRvaS5vcmcvMTAuMTI0Mi9kbW0uMDI5OTY3 |
| id |
ai-49-aHR0cDovL2R4LmRvaS5vcmcvMTAuMTI0Mi9kbW0uMDI5OTY3 |
| institution |
DE-Brt1 DE-Zwi2 DE-D161 DE-Gla1 DE-Zi4 DE-15 DE-Pl11 DE-Rs1 DE-105 DE-14 DE-Ch1 DE-L229 DE-D275 DE-Bn3 |
| imprint |
The Company of Biologists, 2018 |
| imprint_str_mv |
The Company of Biologists, 2018 |
| issn |
1754-8403 1754-8411 |
| issn_str_mv |
1754-8403 1754-8411 |
| language |
English |
| mega_collection |
The Company of Biologists (CrossRef) |
| match_str |
pane2018tbx1repressesmef2cgeneexpressionandiscorrelatedwithhistone3deacetylationoftheanteriorheartfieldenhancer |
| publishDateSort |
2018 |
| publisher |
The Company of Biologists |
| recordtype |
ai |
| record_format |
ai |
| series |
Disease Models & Mechanisms |
| source_id |
49 |
| title |
Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer |
| title_unstemmed |
Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer |
| title_full |
Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer |
| title_fullStr |
Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer |
| title_full_unstemmed |
Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer |
| title_short |
Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer |
| title_sort |
tbx1 represses <i>mef2c</i> gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer |
| topic |
General Biochemistry, Genetics and Molecular Biology Immunology and Microbiology (miscellaneous) Medicine (miscellaneous) Neuroscience (miscellaneous) |
| url |
http://dx.doi.org/10.1242/dmm.029967 |
| publishDate |
2018 |
| physical |
|
| description |
<jats:title>ABSTRACT</jats:title>
<jats:p>The TBX1 gene is haploinsufficient in 22q11.2 deletion syndrome (22q11.2DS), and genetic evidence from human patients and mouse models points to a major role of this gene in the pathogenesis of this syndrome. Tbx1 can activate and repress transcription, and previous work has shown that one of its functions is to negatively modulate cardiomyocyte differentiation. Tbx1 occupies the anterior heart field (AHF) enhancer of the Mef2c gene, which encodes a key cardiac differentiation transcription factor. Here, we show that increased dosage of Tbx1 correlates with downregulation of Mef2c expression and reduced acetylation of its AHF enhancer in cultured mouse myoblasts. Consistently, 22q11.2DS-derived and in vitro-differentiated human induced pluripotent stem cells (hiPSCs) expressed higher levels of MEF2C and showed increased AHF acetylation, compared with hiPSCs from a healthy donor. Most importantly, we show that in mouse embryos, loss of Tbx1 enhances the expression of the Mef2c-AHF-Cre transgene in a specific region of the splanchnic mesoderm, and in a dosage-dependent manner, providing an in vivo correlate of our cell culture data. These results indicate that Tbx1 regulates the Mef2c AHF enhancer by inducing histone deacetylation.</jats:p> |
| container_issue |
9 |
| container_start_page |
0 |
| container_title |
Disease Models & Mechanisms |
| container_volume |
11 |
| format_de105 |
Article, E-Article |
| format_de14 |
Article, E-Article |
| format_de15 |
Article, E-Article |
| format_de520 |
Article, E-Article |
| format_de540 |
Article, E-Article |
| format_dech1 |
Article, E-Article |
| format_ded117 |
Article, E-Article |
| format_degla1 |
E-Article |
| format_del152 |
Buch |
| format_del189 |
Article, E-Article |
| format_dezi4 |
Article |
| format_dezwi2 |
Article, E-Article |
| format_finc |
Article, E-Article |
| format_nrw |
Article, E-Article |
| _version_ |
1792338597932695559 |
| geogr_code |
not assigned |
| last_indexed |
2024-03-01T15:34:46.885Z |
| geogr_code_person |
not assigned |
| openURL |
url_ver=Z39.88-2004&ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fvufind.svn.sourceforge.net%3Agenerator&rft.title=Tbx1+represses+Mef2c+gene+expression+and+is+correlated+with+histone+3+deacetylation+of+the+anterior+heart+field+enhancer&rft.date=2018-09-01&genre=article&issn=1754-8403&volume=11&issue=9&jtitle=Disease+Models+%26+Mechanisms&atitle=Tbx1+represses+%3Ci%3EMef2c%3C%2Fi%3E+gene+expression+and+is+correlated+with+histone+3+deacetylation+of+the+anterior+heart+field+enhancer&aulast=Baldini&aufirst=Antonio&rft_id=info%3Adoi%2F10.1242%2Fdmm.029967&rft.language%5B0%5D=eng |
| SOLR | |
| _version_ | 1792338597932695559 |
| author | Pane, Luna Simona, Fulcoli, Filomena Gabriella, Cirino, Andrea, Altomonte, Alessandra, Ferrentino, Rosa, Bilio, Marchesa, Baldini, Antonio |
| author_facet | Pane, Luna Simona, Fulcoli, Filomena Gabriella, Cirino, Andrea, Altomonte, Alessandra, Ferrentino, Rosa, Bilio, Marchesa, Baldini, Antonio, Pane, Luna Simona, Fulcoli, Filomena Gabriella, Cirino, Andrea, Altomonte, Alessandra, Ferrentino, Rosa, Bilio, Marchesa, Baldini, Antonio |
| author_sort | pane, luna simona |
| container_issue | 9 |
| container_start_page | 0 |
| container_title | Disease Models & Mechanisms |
| container_volume | 11 |
| description | <jats:title>ABSTRACT</jats:title> <jats:p>The TBX1 gene is haploinsufficient in 22q11.2 deletion syndrome (22q11.2DS), and genetic evidence from human patients and mouse models points to a major role of this gene in the pathogenesis of this syndrome. Tbx1 can activate and repress transcription, and previous work has shown that one of its functions is to negatively modulate cardiomyocyte differentiation. Tbx1 occupies the anterior heart field (AHF) enhancer of the Mef2c gene, which encodes a key cardiac differentiation transcription factor. Here, we show that increased dosage of Tbx1 correlates with downregulation of Mef2c expression and reduced acetylation of its AHF enhancer in cultured mouse myoblasts. Consistently, 22q11.2DS-derived and in vitro-differentiated human induced pluripotent stem cells (hiPSCs) expressed higher levels of MEF2C and showed increased AHF acetylation, compared with hiPSCs from a healthy donor. Most importantly, we show that in mouse embryos, loss of Tbx1 enhances the expression of the Mef2c-AHF-Cre transgene in a specific region of the splanchnic mesoderm, and in a dosage-dependent manner, providing an in vivo correlate of our cell culture data. These results indicate that Tbx1 regulates the Mef2c AHF enhancer by inducing histone deacetylation.</jats:p> |
| doi_str_mv | 10.1242/dmm.029967 |
| facet_avail | Online, Free |
| finc_class_facet | Medizin |
| format | ElectronicArticle |
| format_de105 | Article, E-Article |
| format_de14 | Article, E-Article |
| format_de15 | Article, E-Article |
| format_de520 | Article, E-Article |
| format_de540 | Article, E-Article |
| format_dech1 | Article, E-Article |
| format_ded117 | Article, E-Article |
| format_degla1 | E-Article |
| format_del152 | Buch |
| format_del189 | Article, E-Article |
| format_dezi4 | Article |
| format_dezwi2 | Article, E-Article |
| format_finc | Article, E-Article |
| format_nrw | Article, E-Article |
| geogr_code | not assigned |
| geogr_code_person | not assigned |
| id | ai-49-aHR0cDovL2R4LmRvaS5vcmcvMTAuMTI0Mi9kbW0uMDI5OTY3 |
| imprint | The Company of Biologists, 2018 |
| imprint_str_mv | The Company of Biologists, 2018 |
| institution | DE-Brt1, DE-Zwi2, DE-D161, DE-Gla1, DE-Zi4, DE-15, DE-Pl11, DE-Rs1, DE-105, DE-14, DE-Ch1, DE-L229, DE-D275, DE-Bn3 |
| issn | 1754-8403, 1754-8411 |
| issn_str_mv | 1754-8403, 1754-8411 |
| language | English |
| last_indexed | 2024-03-01T15:34:46.885Z |
| match_str | pane2018tbx1repressesmef2cgeneexpressionandiscorrelatedwithhistone3deacetylationoftheanteriorheartfieldenhancer |
| mega_collection | The Company of Biologists (CrossRef) |
| physical | |
| publishDate | 2018 |
| publishDateSort | 2018 |
| publisher | The Company of Biologists |
| record_format | ai |
| recordtype | ai |
| series | Disease Models & Mechanisms |
| source_id | 49 |
| spelling | Pane, Luna Simona Fulcoli, Filomena Gabriella Cirino, Andrea Altomonte, Alessandra Ferrentino, Rosa Bilio, Marchesa Baldini, Antonio 1754-8411 1754-8403 The Company of Biologists General Biochemistry, Genetics and Molecular Biology Immunology and Microbiology (miscellaneous) Medicine (miscellaneous) Neuroscience (miscellaneous) http://dx.doi.org/10.1242/dmm.029967 <jats:title>ABSTRACT</jats:title> <jats:p>The TBX1 gene is haploinsufficient in 22q11.2 deletion syndrome (22q11.2DS), and genetic evidence from human patients and mouse models points to a major role of this gene in the pathogenesis of this syndrome. Tbx1 can activate and repress transcription, and previous work has shown that one of its functions is to negatively modulate cardiomyocyte differentiation. Tbx1 occupies the anterior heart field (AHF) enhancer of the Mef2c gene, which encodes a key cardiac differentiation transcription factor. Here, we show that increased dosage of Tbx1 correlates with downregulation of Mef2c expression and reduced acetylation of its AHF enhancer in cultured mouse myoblasts. Consistently, 22q11.2DS-derived and in vitro-differentiated human induced pluripotent stem cells (hiPSCs) expressed higher levels of MEF2C and showed increased AHF acetylation, compared with hiPSCs from a healthy donor. Most importantly, we show that in mouse embryos, loss of Tbx1 enhances the expression of the Mef2c-AHF-Cre transgene in a specific region of the splanchnic mesoderm, and in a dosage-dependent manner, providing an in vivo correlate of our cell culture data. These results indicate that Tbx1 regulates the Mef2c AHF enhancer by inducing histone deacetylation.</jats:p> Tbx1 represses <i>Mef2c</i> gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer Disease Models & Mechanisms |
| spellingShingle | Pane, Luna Simona, Fulcoli, Filomena Gabriella, Cirino, Andrea, Altomonte, Alessandra, Ferrentino, Rosa, Bilio, Marchesa, Baldini, Antonio, Disease Models & Mechanisms, Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer, General Biochemistry, Genetics and Molecular Biology, Immunology and Microbiology (miscellaneous), Medicine (miscellaneous), Neuroscience (miscellaneous) |
| title | Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer |
| title_full | Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer |
| title_fullStr | Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer |
| title_full_unstemmed | Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer |
| title_short | Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer |
| title_sort | tbx1 represses <i>mef2c</i> gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer |
| title_unstemmed | Tbx1 represses Mef2c gene expression and is correlated with histone 3 deacetylation of the anterior heart field enhancer |
| topic | General Biochemistry, Genetics and Molecular Biology, Immunology and Microbiology (miscellaneous), Medicine (miscellaneous), Neuroscience (miscellaneous) |
| url | http://dx.doi.org/10.1242/dmm.029967 |