Browse
Submit Data
Databases
API
Help

Dataset Information

0 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

A mouse model of rhabdomyosarcoma originating from the adipocyte lineage

ABSTRACT: Activation of Sonic Hedgehog signaling through expression of a constitutively active Smoothened allele under control of an aP2 adipocyte-restricted transgene in mice gives rise to aggressive skeletal muscle tumors that display the histologic and molecular characteristics of human embryonal rhabdomyosarcoma with high penetrance.

ORGANISM(S): Mus musculus

PROVIDER: GSE40359 | GEO | 2012/10/22

SECONDARY ACCESSION(S): PRJNA173689

REPOSITORIES: GEO

ACCESS DATA

Json Xml

Dataset's files

Source:

			Action	DRS
		Other

Items per page:

1 - 1 of 1

Similar Datasets

A mouse model of rhabdomyosarcoma originating from the adipocyte lineage

Project description:Activation of Sonic Hedgehog signaling through expression of a constitutively active Smoothened allele under control of an aP2 adipocyte-restricted transgene in mice gives rise to aggressive skeletal muscle tumors that display the histologic and molecular characteristics of human embryonal rhabdomyosarcoma with high penetrance. Conditional mouse models of embryonal rhabdomyosarcoma were created by activation of the oncogenic SmoM2 allele by both the non-muscle aP2-Cre or myotube Myogenin-Cre.

2012-10-22 | E-GEOD-40359 | biostudies-arrayexpress

Mouse Models of Alveolar/Embryonal Rhabdomyosarcoma & Spindle Cell Sarcomas

Project description:A series of conditional mouse models of embryonal rhabdomyosarcoma, alveolar rhabdomyosarcoma and spindle cell sarcoma were generated and validated for relavence to corresponding human cancers. Conditional mouse models of embryonal rhabdomyosarcoma, alveolar rhabdomyosarcoma and spindle cell sarcoma were created by activation or deletion of Pax3:Fkhr, p53, Ptch1 or Rb1 genes.

2011-02-17 | E-GEOD-22520 | biostudies-arrayexpress

Methylation profiling reveals novel molecular classes of rhabdomyosarcoma

Project description:Rhabdomyosarcomas (RMS) represent a family of aggressive soft tissue sarcomas that present in both the pediatric and adult setting. Pathologic risk stratification for RMS has been based on histologic subtype, with poor outcomes observed in alveolar rhabdomyosarcoma (ARMS) and adult-type pleomorphic rhabdomyosarcoma (PRMS) compared to embryonal rhabdomyosarcoma (ERMS). Recent genomic sequencing studies have expanded the spectrum of RMS, with several new molecularly defined entities, including fusion-driven spindle cell/sclerosing rhabdomyosarcoma (SC/SRMS) and MYOD1-mutant SC/SRMS. Comprehensive genomic analysis has previously defined the mutational and copy number spectrum for the more common ERMS and ARMS, as well as revealed corresponding methylation signatures. In contrast, genetic and epigenetic correlates have not been defined for the rare SC/SRMS or PRMS histologic subtypes. Herein, we present genomic sequencing, copy number analysis, and methylation profiling of the largest cohort of molecularly characterized RMS samples to date. We identified two novel methylation subtypes, one having SC/SRMS histology and defined by MYOD1 p. L122R mutations and the other matching adult type PRMS. Selected tumors from adolescent patients grouped with the PRMS methylation class, expanding the age range of these rare tumors. Pediatric patients in the MYOD1-mutant group, as well as those clustering with PRMS, appear to have poor overall survival.

2021-11-24 | GSE167059 | GEO

Gene Expression in Human Rhabdomyosarcoma

Project description:Gene expression analysis identified unique transcriptional changes in rhabdomyosarcoma when compared with normal muscle. Our studies focused on a gene called VANGL2 that regulated growth and self-renewal in embryonal rhabdomyosarcoma.

2018-03-06 | GSE108022 | GEO

pediatric sarcoma: rhabdomyosarcoma & Ewing's sarcoma

Project description:Affymetrx U95Av2 GeneChip hybridizations, RNA extracted from primary human pediatric alveolar (aRMS) and embryonal (eRMS) rhabdomyosarcoma, plus primary pediatric Ewing's sarcoma (EWS) Keywords = pediatric sarcoma Keywords = Ewing's sarcoma Keywords = rhabdomyosarcoma Keywords: other

2004-03-13 | GSE967 | GEO

Mouse Models of Alveolar/Embryonal Rhabdomyosarcoma & Spindle Cell Sarcomas

2011-02-17 | GSE22520 | GEO

SNP array data for embryonal rhabdomyosarcoma in a patient with Costello syndrome

Project description:Affymetrix SNP array data for embryonal rhabdomyosarcoma in a patient with Costello syndrome

2015-06-12 | GSE41297 | GEO

A mouse model of rhabdomyosarcoma originating from the adipocyte lineage.

Project description:Rhabdomyosarcoma (RMS) is an aggressive skeletal muscle-lineage tumor composed of malignant myoblasts that fail to exit the cell cycle and are blocked from fusing into syncytial muscle. Rhabdomyosarcoma includes two histolopathologic subtypes: alveolar rhabdomyosarcoma, driven by the fusion protein PAX3-FOXO1 or PAX7-FOXO1, and embryonal rhabdomyosarcoma (ERMS), which is genetically heterogeneous. Here, we show that adipocyte-restricted activation of Sonic hedgehog signaling through expression of a constitutively active Smoothened allele in mice gives rise to aggressive skeletal muscle tumors that display the histologic and molecular characteristics of human ERMS with high penetrance. Our findings suggest that adipocyte progenitors can be a cell of origin for Sonic hedgehog-driven ERMS, showing that RMS can originate from nonskeletal muscle precursors.

| S-EPMC3479681 | biostudies-literature

SNP array data for embryonal rhabdomyosarcoma in a patient with Costello syndrome

Project description:Affymetrix SNP array data for embryonal rhabdomyosarcoma in a patient with Costello syndrome Affymetrix SNP array was performed according to the manufacturer's directions on DNA extracted from a fresh-frozen tumor sample.

2015-06-12 | E-GEOD-41297 | biostudies-arrayexpress

Serial xenotransplantation in NSG mice promotes a hybrid epithelial/mesenchymal gene expression signature and stemness in rhabdomyosarcoma cells

Project description:NSTS-11 cell line derived from a primary embryonal rhabdomyosarcoma was used for serial xenotransplantation in NSG mice in vivo. Subsequently, three cell lines, LTB1, LTB5, and LTB24, were derived from primary, secondary, and tertiary xenograft tumors, respectively. Gene expression profiling of all four cell lines was performed to identify changes in gene expression over the course of serial xenotransplantation, which was associated with a gradual increase of stemness of embryonal rhabdomyosarcoma cells. For each of the xenograft-derived cell lines, the parental NSTS-11 cell line served as a reference control.

2019-04-30 | E-MTAB-7664 | biostudies-arrayexpress

OmicsDI is part of the ELIXIR infrastructure

OmicsDI is an Elixir interoperability service. Learn more ›

Tweets

OmicsDI Databases

PRIDE
PeptideAtlas
MassIVE
JPOST Repository
Physiome Model Repository

EGA
EVA
ENA
LINCS
PAXDB
Cell Collective

MetaboLights
Metabolomics Workbench
MetabolomeExpress
GNPS
BioModels
FAIRDOMHub

ArrayExpress
dbGaP
ExpressionAtlas
GEO
NODE

Information

Databases
Help
API
Contact us
Code on GitHub
Terms of use
Submit Data