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Series GSE84504 Query DataSets for GSE84504
Status Public on Apr 09, 2017
Title Modeling Trilateral Retinoblastoma using Human Embryonic Stem Cells
Organism Homo sapiens
Experiment type Expression profiling by high throughput sequencing
Summary Human embryonic stem cells (hESCs) provide a platform for understanding mechanisms underlying diseases and finding ways to treat them. Trilateral retinoblastoma (TRb) is a condition in which retinoblastoma, the most common congenital intraocular malignancy, is associated with an intracranial neural tumor. This mostly fatal disease is caused by biallelic inactivation of the retinoblastoma-1 (RB1) gene, transcribing pRB. Using CRISPR-Cas9 we generated RB1-null hESCs, an embryonic lethal condition. These cells display distinct gene expression patterns with alterations in pRB targets, and mitochondrial phenotypes similar to those of poorly differentiated retinoblastomas. RB1–/– hESC produce extremely large teratomas, with neural expansions similar to those of TRb tumors. Analysis of these tumors revealed a potentially novel role of pRB in ectoderm differentiation, acting through ZEB1. Lastly, RB1–/– cells are significantly sensitive to carboplatin, a chemotherapy used to treat TRb, suggesting our model can be used to screen novel treatments for the disease.
Overall design Analysis of RB1–/– human embryonic cell lines and teratomas initiated from them
Contributor(s) Avior Y, Benvenisty N
Citation(s) 28392220
Submission date Jul 18, 2016
Last update date May 15, 2019
Contact name Yishai Avior
Phone +972-2-6584803
Organization name Hebrew University of Jeursalem
Department Genetics
Lab Nissim Benvenisty
Street address Edmond J. Safra Campus, Givat Ram
City Jerusalem
State/province Israel
ZIP/Postal code 91904
Country Israel
Platforms (1)
GPL18573 Illumina NextSeq 500 (Homo sapiens)
Samples (10)
GSM2238613 Control A (CSES7)
GSM2238614 Control B (CSES7, Cas9 No guide)
GSM2238615 RB1-/- A
BioProject PRJNA329518
SRA SRP078823

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Supplementary file Size Download File type/resource
GSE84504_Teratoma_RB1.txt.gz 877.1 Kb (ftp)(http) TXT
GSE84504_hESC_RB1.txt.gz 627.9 Kb (ftp)(http) TXT
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