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EW-8Homo sapiens (Human)Cancer cell line

Also known as: SJRH1, SJRH-1, Rh-1, Rh1 RH-1, RH1, EW8(Rh1), EW8

🤖 AI SummaryBased on 10 publications

Quick Overview

Human rhabdomyosarcoma cell line with alveolar subtype and PAX3-FKHR fusion.

Detailed Summary

The EW-8 (CVCL_1658) cell line is a human rhabdomyosarcoma cell line derived from an alveolar subtype, characterized by the presence of the PAX3-FKHR fusion gene. This fusion is a hallmark of alveolar rhabdomyosarcoma and is associated with aggressive tumor behavior. The cell line exhibits a t(2;13)(q35;q14) chromosomal translocation, which leads to the formation of the chimeric PAX3-FKHR transcript. The cell line is used in research to study the molecular mechanisms of rhabdomyosarcoma, including the role of the PAX3-FKHR fusion in tumorigenesis and potential therapeutic targets. It is also utilized in drug screening studies to evaluate the efficacy of anticancer agents against this specific genetic alteration.

Research Applications

Molecular mechanisms of rhabdomyosarcomaPAX3-FKHR fusion gene studiesDrug screening for anticancer agents

Key Characteristics

PAX3-FKHR fusion genet(2;13)(q35;q14) chromosomal translocationAlveolar subtype

Generated on 6/17/2025

Basic Information

Database ID	CVCL_1658
Species	Homo sapiens (Human)
Tissue Source	Bone marrow[UBERON:UBERON_0002371]

Donor Information

Age Category	Pediatric
Sex	Male
Subtype Features	EWS-FLI

Disease Information

Disease	Ewing sarcoma
Lineage	Bone
Subtype	Ewing Sarcoma
OncoTree Code	ES

DepMap Information

Source Type	Academic lab
Source ID	ACH-000499_source

Known Sequence Variations

Type	Gene/Protein	Description	Zygosity	Note	Source
MutationSimple	TP53	p.Tyr220Cys (c.659A>G)	Unspecified	-	PubMed=21173094
MutationSimple	KIT	p.Arg55Trp (c.163A>T)	Heterozygous	-	Unknown, Unknown
Gene fusion	EWSR1	EWSR1-FLI1, EWS-FLI1	-	Type 1 fusion	PubMed=15150091

Haplotype Information (STR Profile)

Short Tandem Repeat (STR) profile for cell line authentication.

Amelogenin

X,Y

CSF1PO

11

D13S317

10

D16S539

13

D18S51

15

D19S433

13,15.2

D21S11

29,30

D2S1338

16,19

D3S1358

11,18

D5S818

11

D7S820

8,12

D8S1179

14,16

FGA

22,23

Penta D

9,13

Penta E

5,16

TH01

9.3

TPOX

8

vWA

17

Gene Expression Profile

Gene expression levels and statistical distribution

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Full DepMap dataset with combined data across cell lines

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Publications

Pan-cancer proteomic map of 949 human cell lines.";

Robinson P.J., Zhong Q., Garnett M.J., Reddel R.R.

Cancer Cell 40:835-849.e8(2022).

Next-generation characterization of the Cancer Cell Line Encyclopedia.

Sellers W.R.

Nature 569:503-508(2019).

An interactive resource to probe genetic diversity and estimated ancestry in cancer cell lines.

Dutil J., Chen Z.-H., Monteiro A.N.A., Teer J.K., Eschrich S.A.

Cancer Res. 79:1263-1273(2019).

A landscape of pharmacogenomic interactions in cancer.";

Wessels L.F.A., Saez-Rodriguez J., McDermott U., Garnett M.J.

Cell 166:740-754(2016).

Sarcoma cell line screen of oncology drugs and investigational agents identifies patterns associated with gene and microRNA expression.

Harris E., Monks A., Morris J.

Mol. Cancer Ther. 14:2452-2462(2015).

Parallel genome-scale loss of function screens in 216 cancer cell lines for the identification of context-specific genetic dependencies.

Golub T.R., Root D.E., Hahn W.C.

Sci. Data 1:140035-140035(2014).

The genomic landscape of the Ewing sarcoma family of tumors reveals recurrent STAG2 mutation.

Catchpoole D., Llombart-Bosch A., Waldman T., Khan J.

PLoS Genet. 10:E1004475-E1004475(2014).

Human rhabdomyosarcoma cell lines for rhabdomyosarcoma research: utility and pitfalls.

Linardic C.M.

Front. Oncol. 3:183.1-183.12(2013).

Characterization of genetic lesions in rhabdomyosarcoma using a high-density single nucleotide polymorphism array.

Ogawa S.

Cancer Sci. 104:856-864(2013).

Signatures of mutation and selection in the cancer genome.";

Deloukas P., Yang F.-T., Campbell P.J., Futreal P.A., Stratton M.R.

Nature 463:893-898(2010).

SK-NEP-1 and Rh1 are Ewing family tumor lines.";

Houghton P.J.

Pediatr. Blood Cancer 50:703-706(2008).

Pediatric rhabdomyosarcoma cell lines are resistant to Fas-induced apoptosis and highly sensitive to TRAIL-induced apoptosis.

Petak I., Douglas L., Tillman D.M., Vernes R., Houghton J.A.

Clin. Cancer Res. 6:4119-4127(2000).

Fusion of a fork head domain gene to PAX3 in the solid tumour alveolar rhabdomyosarcoma.

Rauscher F.J. 3rd, Emanuel B.S., Rovera G., Barr F.G.

Nat. Genet. 5:230-235(1993).

Detection of the t(2;13)(q35;q14) and PAX3-FKHR fusion in alveolar rhabdomyosarcoma by fluorescence in situ hybridization.

Biegel J.A., Nycum L.M., Valentine V.A., Barr F.G., Shapiro D.N.

Genes Chromosomes Cancer 12:186-192(1995).

Systematic multi-omics profiling of Ewing sarcoma cell lines.";

Orth M.F.

Thesis PhD (2021); Ludwig Maximilians University of Munich; Munich; Germany.