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Series GSE248120 Query DataSets for GSE248120
Status Public on Jan 24, 2024
Title Unfolded protein Response is a major hurdle in direct neuronal reprogramming of human astrocytes [Bulk RNA-seq]
Organism Homo sapiens
Experiment type Expression profiling by high throughput sequencing
Summary Mitochondria account for essential cellular pathways, from ATP production to nucleotide metabolism, and their deficits lead to neurological disorders and contribute to the onset of age-related diseases. Direct neuronal reprogramming aims at replacing neurons lost in such conditions, but almost nothing is known about the impact of mitochondrial dysfunction in human cell direct reprogramming. Here we explore the defects and how to improve the neuronal reprogramming of iPSC-derived astrocytes carrying mutations in NDUFS4 gene, important for Complex I and associated to Leigh Sydrome. This led to the identification of the unfolded protein response as a major hurdle in the direct neuronal conversion of not only patient, but also control astrocytes and fibroblasts. Our data highlight mitochondria-ER stress-mediated inhibition of translation as a key hurdle in direct neuronal reprogramming, emphasizing how disease modelling using patient cells can contribute to unravel novel pathways in human glia cell reprogramming.
 
Overall design human IPSCs from control or patients with NDUFS4 mutations were differentiated into proliferating and non-proliferating astrocytes. Three control and 3 patients lines were analyzed. Per each line, 3 biological replicates were collected at 3 stages: IPSC, proliferating astrocyte and non-proliferating astrocytes.

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Raw data will be deposited in GHGA when the database is available
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Contributor(s) Sonsalla G, Masserdotti G, Smialowski P, Prokisch H
Citation missing Has this study been published? Please login to update or notify GEO.
Submission date Nov 17, 2023
Last update date Jan 24, 2024
Contact name Giacomo Masserdotti
E-mail(s) giacomo.masserdotti@helmholtz-munich.de
Organization name Helmholtz Zentrum
Department ISF
Street address Groshaderner Strasse 9
City Planegg-Martinstied
ZIP/Postal code 82152
Country Germany
 
Platforms (1)
GPL18460 Illumina HiSeq 1500 (Homo sapiens)
Samples (55)
GSM7906112 control_HMGU-1_iPSC_1
GSM7906113 control_ULERi82a-R1-002_pAstro_2
GSM7906114 control_HMGU-1_nonpAstro_3
This SubSeries is part of SuperSeries:
GSE248129 Unfolded protein Response is a major hurdle in direct neuronal reprogramming of human astrocytes
Relations
BioProject PRJNA1041800

Download family Format
SOFT formatted family file(s) SOFTHelp
MINiML formatted family file(s) MINiMLHelp
Series Matrix File(s) TXTHelp

Supplementary file Size Download File type/resource
GSE248120_Bulk_RNAseq.csv.gz 2.2 Mb (ftp)(http) CSV
Raw data not provided for this record

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