Jariyasakulroj et al. 2023 (PRJNA926978)

General Details

Title Ribosome biogenesis controls suture mesenchymal stem cell fate via selective regulation ofcomplement pathway in to prevent craniosynostosis [Ribo-seq]
Organism
Number of Samples 4
Release Date 2023/01/24 00:00
Sequencing Types
Protocol Details

Study Links

Repository Details

SRA SRP418884
ENA SRP418884
GEO GSE223615
BioProject PRJNA926978

Publication

Title
Authors Jariyasakulroj S, Zhang W, Bai J, Zhang M, Lu Z, Chen JF
Journal Stem cell reports
Publication Date 2023 Dec 12
Abstract Disruption of global ribosome biogenesis selectively affects craniofacial tissues with unclear mechanisms. Craniosynostosis is a congenital craniofacial disorder characterized by premature fusion of cranial suture(s) with loss of suture mesenchymal stem cells (MSCs). Here we focused on ribosomopathy disease gene Snord118, which encodes a small nucleolar RNA (snoRNA), to genetically disturb ribosome biogenesis in suture MSCs using mouse and human induced pluripotent stem cell (iPSC) models. Snord118 depletion exhibited p53 activation, increased cell death, reduced proliferation, and premature osteogenic differentiation of MSCs, leading to suture growth and craniosynostosis defects. Mechanistically, Snord118 deficiency causes translational dysregulation of ribosomal proteins and downregulation of complement pathway genes. Further complement pathway disruption by knockout of complement C3a receptor 1 (C3ar1) exacerbated MSC and suture defects in mutant mice, whereas activating the complement pathway rescued MSC cell fate and suture growth defects. Thus, ribosome biogenesis controls MSC fate via the complement pathway to prevent craniosynostosis. Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.
PMC PMC10724072
PMID 37977145
DOI
Run Accession Study Accession Scientific Name Cell Line Library Type Treatment GWIPS-viz Trips-Viz Reads BAM BigWig (F) BigWig (R)
SRR23204134 PRJNA926978 Homo sapiens human iPS derived mesenchymal stem cellsNA_mesenchymal stem cells Ribo-Seq 0.0
SRR23204133 PRJNA926978 Homo sapiens human iPS derived mesenchymal stem cellsNA_mesenchymal stem cells Ribo-Seq 0.0
SRR23204132 PRJNA926978 Homo sapiens human iPS derived mesenchymal stem cellsNA_mesenchymal stem cells Ribo-Seq 0.0
SRR23204131 PRJNA926978 Homo sapiens human iPS derived mesenchymal stem cellsNA_mesenchymal stem cells Ribo-Seq 0.0
Run Accession Study Accession Scientific Name Cell Line Library Type Treatment GWIPS-viz Trips-Viz Reads BAM BigWig (F) BigWig (R)

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