Genomics

Dataset Information

0

The deletion of the Williams-Beuren Syndrome critical region dysregulate the expression of miR379-410 miRNA members in human isogenic iNeurons


ABSTRACT: While psychiatric disorders (e.g., schizophrenia) and autism spectrum disorders (ASD) are typically associated with a deficit in social behavior, the opposite trait of hypersociability is exhibited by individuals with specific neurodevelopmental disorders, e.g., Angelman Syndrome (AS) and Williams-Beuren Syndrome (WBS). We have recently reported that the deletion of the miR379-410 cluster in mice led to hypersocial behavior. To study the roles of this miRNA cluster in the context of WBS, we sent for smallRNA sequencing RNA isolated from isogenic human iPSC-derived neurons harboring a deletion present in Williams-Beuren-Syndrome patients (7q11.23). Specifically, we found that members of the miR379-410 cluster were strikingly overrepresented among downregulated miRNAs in iNeurons harboring a deletion of the WBS critical region. Thus, we obtained the first evidence for the pathophysiological significance of the miR379-410 miRNA cluster in the context of WBS. We conclude that targeting this novel pathway could have therapeutic potential for WBS and other neurodevelopmental conditions characterized by social impairments.

ORGANISM(S): Homo sapiens

PROVIDER: GSE247766 | GEO | 2023/11/15

REPOSITORIES: GEO

Dataset's files

Source:
Action DRS
Other
Items per page:
1 - 1 of 1

Similar Datasets

2011-01-31 | GSE16715 | GEO
2024-10-17 | PXD035276 | Pride
2024-01-26 | PXD036770 | Pride
2011-01-31 | E-GEOD-16715 | biostudies-arrayexpress
2010-09-09 | GSE18188 | GEO
2024-10-17 | PXD038156 | Pride
2010-09-09 | E-GEOD-18188 | biostudies-arrayexpress
2023-05-03 | GSE180040 | GEO
2014-12-15 | E-GEOD-63028 | biostudies-arrayexpress
2014-12-15 | E-GEOD-63057 | biostudies-arrayexpress