Proteomics

Dataset Information

0

Targeting microRNA-dependent control of X chromosome inactivation improves the Rett Syndrome phenotype

ABSTRACT: The X chromosome inactivation (XCI) is induced by Xist long non-coding RNA and protein-coding genes. However, the small non-coding RNA function in XCI remains unidentified. Our genome-wide, loss-of-function CRISPR/Cas9 screen in female fibroblasts examines the role of microRNA (miRNAs) in XCI. A striking finding is the identification of miR106a among the top candidates from 46 the screen. Loss of miR106a is accompanied by altered Xist interactome, leading to dissociation and destabilization of Xist. The XCI interference via miR106a inhibition has therapeutic implications for Rett syndrome (RTT) girls with a defective X-linked MECP2 gene. Here, we discovered that the genetic inhibition of miR106a significantly improves several facets of RTT pathology: it increases the life span, enhances locomotor activity and exploratory behavior, and diminishes breathing variabilities. Our results suggest that miR106a targeting offers a feasible therapeutic strategy for RTT and other monogenic X-linked neurodevelopmental disorders.

INSTRUMENT(S):

ORGANISM(S): Mus Musculus (mouse)

SUBMITTER: Robert Maxwell  

LAB HEAD: Sanchita Bhatnagar

PROVIDER: PXD064749 | Pride | 2026-02-09

REPOSITORIES: Pride

Json Xml

Dataset's files

Source:
Action DRS
SongL_Sample1HpH_2of4.mgf Mgf
SongL_Sample1HpH_2of4.raw Raw
SongL_Sample1HpH_4of4_r3.mgf Mgf
SongL_Sample1HpH_4of4_r3.raw Raw
SongL_Sample1_HpH_1of4.mgf Mgf
Items per page:
1 - 5 of 66
altmetric image

Publications

X chromosome inactivation (XCI) is induced by Xist long non-coding RNA and protein-coding genes. However, the role of small non-coding RNA function in XCI remains unidentified. Our genome-wide, loss-of-function CRISPR/Cas9 screen in female fibroblasts identified microRNAs (miRNAs) as regulators of XCI. A striking finding is the identification of miR106a among the top candidates from the screen. Loss of miR106a is accompanied by altered Xist interactome, leading to dissociation and destabilizatio  ...[more]

Similar Datasets

Genomics ChIP-sequencing (ChIP-seq) experiments on female ESCs expressing V5-tagged REX1 and control wild-type Embryonic stem cells (ESCs)
2012-06-12 | E-GEOD-36417 | biostudies-arrayexpress
Transcriptomics Transcriptomic analysis of RTT neurons expressing miR106a-specifc sponge
2025-05-01 | GSE261876 | GEO
Proteomics SPEN is a molecular integrator that bridges Xist with transcriptional and epigenetic control of X-chromosome inactivation
2021-04-19 | PXD015699 | Pride
Proteomics The role of Xist-mediated Polycomb recruitment in the initiation of X-chromosome inactivation
2019-08-29 | PXD014287 | Pride
Genomics Derivation of pre-X inactivation human embryonic stem cells under physiological oxygen concentrations
2010-05-28 | E-GEOD-21141 | biostudies-arrayexpress
Transcriptomics En bloc and segmental deletions of human XIST reveal X chromosome inactivation-involving RNA elements
2019-01-25 | GSE65830 | GEO
Proteomics XIST CHIRP-MS in GM12878 and K562 cell line
2021-03-08 | PXD023516 | Pride
Transcriptomics XIST directly regulates X-linked and autosomal genes in naïve human pluripotent cells [scRNA-seq]
2023-12-15 | GSE241441 | GEO
Other XIST directly regulates X-linked and autosomal genes in naïve human pluripotent cells [RAP-seq_mouse]
2023-12-15 | GSE241440 | GEO
Other XIST directly regulates X-linked and autosomal genes in naïve human pluripotent cells [RAP-seq_human]
2023-12-15 | GSE241439 | GEO