Neuronal genes deregulated in Cornelia de Lange Syndrome respond to removal and re-expression of cohesin
A feature of cohesin mutations in patients with Cornelia de Lange Syndrome (CdLS) is intellectual disability, but the underlying mechanisms have remained obscure. Here the authors show gene expression is deregulated in CdLS neurons and is recapitulated in a mouse model with cohesin depletion, which...
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2021-05-01
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Online Access: | https://doi.org/10.1038/s41467-021-23141-9 |
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doaj-afef53aab6c24336822425f4c1c3c8be2021-05-23T11:13:14ZengNature Publishing GroupNature Communications2041-17232021-05-0112111310.1038/s41467-021-23141-9Neuronal genes deregulated in Cornelia de Lange Syndrome respond to removal and re-expression of cohesinFelix D. Weiss0Lesly Calderon1Yi-Fang Wang2Radina Georgieva3Ya Guo4Nevena Cvetesic5Maninder Kaur6Gopuraja Dharmalingam7Ian D. Krantz8Boris Lenhard9Amanda G. Fisher10Matthias Merkenschlager11Lymphocyte Development Group, Epigenetics Section, MRC London Institute of Medical Sciences, Institute of Clinical Sciences, Faculty of Medicine, Imperial College LondonLymphocyte Development Group, Epigenetics Section, MRC London Institute of Medical Sciences, Institute of Clinical Sciences, Faculty of Medicine, Imperial College LondonMRC London Institute of Medical Sciences, Institute of Clinical Sciences, Faculty of Medicine, Imperial College LondonLymphocyte Development Group, Epigenetics Section, MRC London Institute of Medical Sciences, Institute of Clinical Sciences, Faculty of Medicine, Imperial College LondonLymphocyte Development Group, Epigenetics Section, MRC London Institute of Medical Sciences, Institute of Clinical Sciences, Faculty of Medicine, Imperial College LondonComputational Regulatory Genomics Group, Epigenetics Section, MRC London Institute of Medical Sciences, Institute of Clinical Sciences, Faculty of Medicine, Imperial College LondonDivision of Human Genetics, The Department of Pediatrics, The Children’s Hospital of PhiladelphiaMRC London Institute of Medical Sciences, Institute of Clinical Sciences, Faculty of Medicine, Imperial College LondonDivision of Human Genetics, The Department of Pediatrics, The Children’s Hospital of PhiladelphiaComputational Regulatory Genomics Group, Epigenetics Section, MRC London Institute of Medical Sciences, Institute of Clinical Sciences, Faculty of Medicine, Imperial College LondonLymphocyte Development Group, Epigenetics Section, MRC London Institute of Medical Sciences, Institute of Clinical Sciences, Faculty of Medicine, Imperial College LondonLymphocyte Development Group, Epigenetics Section, MRC London Institute of Medical Sciences, Institute of Clinical Sciences, Faculty of Medicine, Imperial College LondonA feature of cohesin mutations in patients with Cornelia de Lange Syndrome (CdLS) is intellectual disability, but the underlying mechanisms have remained obscure. Here the authors show gene expression is deregulated in CdLS neurons and is recapitulated in a mouse model with cohesin depletion, which can be restored by re-expression of cohesin.https://doi.org/10.1038/s41467-021-23141-9 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Felix D. Weiss Lesly Calderon Yi-Fang Wang Radina Georgieva Ya Guo Nevena Cvetesic Maninder Kaur Gopuraja Dharmalingam Ian D. Krantz Boris Lenhard Amanda G. Fisher Matthias Merkenschlager |
spellingShingle |
Felix D. Weiss Lesly Calderon Yi-Fang Wang Radina Georgieva Ya Guo Nevena Cvetesic Maninder Kaur Gopuraja Dharmalingam Ian D. Krantz Boris Lenhard Amanda G. Fisher Matthias Merkenschlager Neuronal genes deregulated in Cornelia de Lange Syndrome respond to removal and re-expression of cohesin Nature Communications |
author_facet |
Felix D. Weiss Lesly Calderon Yi-Fang Wang Radina Georgieva Ya Guo Nevena Cvetesic Maninder Kaur Gopuraja Dharmalingam Ian D. Krantz Boris Lenhard Amanda G. Fisher Matthias Merkenschlager |
author_sort |
Felix D. Weiss |
title |
Neuronal genes deregulated in Cornelia de Lange Syndrome respond to removal and re-expression of cohesin |
title_short |
Neuronal genes deregulated in Cornelia de Lange Syndrome respond to removal and re-expression of cohesin |
title_full |
Neuronal genes deregulated in Cornelia de Lange Syndrome respond to removal and re-expression of cohesin |
title_fullStr |
Neuronal genes deregulated in Cornelia de Lange Syndrome respond to removal and re-expression of cohesin |
title_full_unstemmed |
Neuronal genes deregulated in Cornelia de Lange Syndrome respond to removal and re-expression of cohesin |
title_sort |
neuronal genes deregulated in cornelia de lange syndrome respond to removal and re-expression of cohesin |
publisher |
Nature Publishing Group |
series |
Nature Communications |
issn |
2041-1723 |
publishDate |
2021-05-01 |
description |
A feature of cohesin mutations in patients with Cornelia de Lange Syndrome (CdLS) is intellectual disability, but the underlying mechanisms have remained obscure. Here the authors show gene expression is deregulated in CdLS neurons and is recapitulated in a mouse model with cohesin depletion, which can be restored by re-expression of cohesin. |
url |
https://doi.org/10.1038/s41467-021-23141-9 |
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