Dysregulation of REST and its target genes impacts the fate of neural progenitor cells in down syndrome

Increasing shreds of evidence suggest that neurogenic-to-gliogenic shift may be critical to the abnormal neurodevelopment observed in individuals with Down syndrome (DS). REST, the Repressor Element-1 Silencing Transcription factor, regulates the differentiation and development of neural cells. Down...

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Main Authors: Tan, Huang, Fakurazi, Sharida, Cheah, Pike-See, Ling, King-Hwa
Format: Article
Language:English
Published: Nature Research 2025
Online Access:http://psasir.upm.edu.my/id/eprint/120219/
http://psasir.upm.edu.my/id/eprint/120219/1/120219.pdf
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author Tan, Huang
Fakurazi, Sharida
Cheah, Pike-See
Ling, King-Hwa
author_facet Tan, Huang
Fakurazi, Sharida
Cheah, Pike-See
Ling, King-Hwa
author_sort Tan, Huang
building UPM Institutional Repository
collection Online Access
description Increasing shreds of evidence suggest that neurogenic-to-gliogenic shift may be critical to the abnormal neurodevelopment observed in individuals with Down syndrome (DS). REST, the Repressor Element-1 Silencing Transcription factor, regulates the differentiation and development of neural cells. Downregulation of REST may lead to defects in post-differentiation neuronal morphology in the brain of the DS fetal. This study aims to elucidate the role of REST in DS-derived NPCs using bioinformatics analyses and laboratory validations. We identified and validated vital REST-targeted DEGs: CD44, TGFB1, FN1, ITGB1, and COL1A1. Interestingly, these genes are involved in neurogenesis and gliogenesis in DS-derived NPCs. Furthermore, we identified nuclear REST loss and the neuroblast marker, DCX, was downregulated in DS human trisomic induced pluripotent stem cells (hiPSCs)-derived NPCs, whereas the glioblast marker, NFIA, was upregulated. Our findings indicate that the loss of REST is critical in the neurogenic-to-gliogenic shift observed in DS-derived NPCs. REST and its target genes may collectively regulate the NPC phenotype.
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spelling upm-1202192025-09-25T07:09:21Z http://psasir.upm.edu.my/id/eprint/120219/ Dysregulation of REST and its target genes impacts the fate of neural progenitor cells in down syndrome Tan, Huang Fakurazi, Sharida Cheah, Pike-See Ling, King-Hwa Increasing shreds of evidence suggest that neurogenic-to-gliogenic shift may be critical to the abnormal neurodevelopment observed in individuals with Down syndrome (DS). REST, the Repressor Element-1 Silencing Transcription factor, regulates the differentiation and development of neural cells. Downregulation of REST may lead to defects in post-differentiation neuronal morphology in the brain of the DS fetal. This study aims to elucidate the role of REST in DS-derived NPCs using bioinformatics analyses and laboratory validations. We identified and validated vital REST-targeted DEGs: CD44, TGFB1, FN1, ITGB1, and COL1A1. Interestingly, these genes are involved in neurogenesis and gliogenesis in DS-derived NPCs. Furthermore, we identified nuclear REST loss and the neuroblast marker, DCX, was downregulated in DS human trisomic induced pluripotent stem cells (hiPSCs)-derived NPCs, whereas the glioblast marker, NFIA, was upregulated. Our findings indicate that the loss of REST is critical in the neurogenic-to-gliogenic shift observed in DS-derived NPCs. REST and its target genes may collectively regulate the NPC phenotype. Nature Research 2025 Article PeerReviewed text en cc_by_nc_nd_4 http://psasir.upm.edu.my/id/eprint/120219/1/120219.pdf Tan, Huang and Fakurazi, Sharida and Cheah, Pike-See and Ling, King-Hwa (2025) Dysregulation of REST and its target genes impacts the fate of neural progenitor cells in down syndrome. Scientific Reports, 15 (1). art. no. 2818. pp. 1-17. ISSN 2045-2322 https://www.nature.com/articles/s41598-025-87314-y?error=cookies_not_supported&code=25271904-06cf-4c36-919f-f75a848d47c9 10.1038/s41598-025-87314-y
spellingShingle Tan, Huang
Fakurazi, Sharida
Cheah, Pike-See
Ling, King-Hwa
Dysregulation of REST and its target genes impacts the fate of neural progenitor cells in down syndrome
title Dysregulation of REST and its target genes impacts the fate of neural progenitor cells in down syndrome
title_full Dysregulation of REST and its target genes impacts the fate of neural progenitor cells in down syndrome
title_fullStr Dysregulation of REST and its target genes impacts the fate of neural progenitor cells in down syndrome
title_full_unstemmed Dysregulation of REST and its target genes impacts the fate of neural progenitor cells in down syndrome
title_short Dysregulation of REST and its target genes impacts the fate of neural progenitor cells in down syndrome
title_sort dysregulation of rest and its target genes impacts the fate of neural progenitor cells in down syndrome
url http://psasir.upm.edu.my/id/eprint/120219/
http://psasir.upm.edu.my/id/eprint/120219/
http://psasir.upm.edu.my/id/eprint/120219/
http://psasir.upm.edu.my/id/eprint/120219/1/120219.pdf