GEO Logo
   NCBI > GEO > Accession DisplayHelp Not logged in | LoginHelp
GEO help: Mouse over screen elements for information.
Series GSE133014 Query DataSets for GSE133014
Status Public on Jan 01, 2020
Title Distinct SOX signature determines neuroblastoma origin and predicts clinical outcome
Organism Homo sapiens
Experiment type Expression profiling by high throughput sequencing
Summary Multimodality treatment of high-risk neuroblastoma can be effective, but up to 50%  of children experience recurrent disease with fatal outcome. Extensive genetic intratumoral heterogeneity and diverse clinical outcomes pose therapeutic challenges in treating children affected by this aggressive disease. Several hypotheses have been proposed to explain the heterogeneity of neuroblastoma, including a possible origin from multipotent neural crest stem cells (NCSCs). Utilizing an in vivo mouse genetics approach, we demonstrate that lineage-restricted sympathoadrenal (SA) progenitors and not NCSCs are the cellular origin of neuroblastoma. Human neuroblastoma tissue is composed of two spatially juxtaposed cell types, neuroblasts and Schwann cells, both cell types derive from neural crest (NC) lineage. This composition mimics the architecture of sympathetic ganglions (SG) as well as of adrenal medulla at the late stage of neural crest (NC)  development. Similarly to SG, SOX10 is restricted to Schwannian cells and is not present in tumorigenic neuroblasts. Transcriptional landscape of Sox genes can serve as paradigmatic model for stage identification along NC lineage development. While early multipotent NCSCs are characterized by the expression of Sox9/Sox10 transcription factors (TF), committed SA progenitor identity is defined by the presence of Sox4/Sox11 TFs. Molecularly, we show that the core transcriptional network that orchestrate neuroblastoma maintenance is highly reminiscent of the SA progenitors. Taken together, the data presented here show that neuroblastoma cells functionally resemble the committed SA progenitors, while lacking specific stem cell program.
Overall design RNA-seq of human neuroblastoma cell lines overexpress or knockdown SOX9
Contributor(s) Yang C, Serra-Roma A, Shakhova O
Citation(s) 32595833
Submission date Jun 19, 2019
Last update date Jul 15, 2020
Contact name giancarlo russo
Organization name ETH/University of Zurich
Department Functional Genomics Center Zurich
Street address winterthurerstrasse 190
City Zurich
State/province Schweiz
ZIP/Postal code 8057
Country Switzerland
Platforms (2)
GPL20301 Illumina HiSeq 4000 (Homo sapiens)
GPL24676 Illumina NovaSeq 6000 (Homo sapiens)
Samples (18)
GSM3898318 IMR5_GFP_1105
GSM3898319 IMR5_SOX9-GFP_1105
GSM3898320 IMR5_GFP_1108
BioProject PRJNA549698
SRA SRP201900

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
GSE133014_RAW.tar 1.4 Mb (http)(custom) TAR (of TXT)
SRA Run SelectorHelp
Raw data are available in SRA
Processed data provided as supplementary file

| NLM | NIH | GEO Help | Disclaimer | Accessibility |
NCBI Home NCBI Search NCBI SiteMap