Developmental loss of MeCP2 from VIP interneurons impairs cortical function and behavior.
basic_science · Level V
Where this comes from
- Record sourced from PubMed, PMID 32343226.
- Also identified by DOI 10.7554/eLife.55639 and PMC identifier 7213975.
- Licence recorded as CC BY.
- The licence permits redistribution, so the abstract is shown in full and the full text is available from the publisher.
Abstract
Rett Syndrome is a devastating neurodevelopmental disorder resulting from mutations in the gene <i>MECP2</i>. Mutations of <i>Mecp2</i> that are restricted to GABAergic cell types largely replicate the behavioral phenotypes associated with mouse models of Rett Syndrome, suggesting a pathophysiological role for inhibitory interneurons. Recent work has suggested that vasoactive intestinal peptide-expressing (VIP) interneurons may play a critical role in the proper development and function of cortical circuits, making them a potential key point of vulnerability in neurodevelopmental disorders. However, little is known about the role of VIP interneurons in Rett Syndrome. Here we find that loss of MeCP2 specifically from VIP interneurons replicates key neural and behavioral phenotypes observed following global <i>Mecp2</i> loss of function.
Medical subject headings
- Interneurons
- Methyl-CpG-Binding Protein 2
- Rett Syndrome
- Vasoactive Intestinal Peptide