Rh30Homo sapiens (Human)Cancer cell line

Also known as: RH30, RH-30, Rh-30, RH30SJ, SJRH-30, SJRH30, SJ-RH30, SJ-Rh 30, SJRH 30, SJCRH30, RC13, RMS-13, RMS 13, RMS13

🤖 AI SummaryBased on 13 publications

Quick Overview

Rh30 is a human alveolar rhabdomyosarcoma cell line used in cancer research.

Detailed Summary

Rh30 is a human alveolar rhabdomyosarcoma cell line derived from a bone marrow metastasis. It is characterized by the presence of the t(2;13)(q35;q14) chromosomal translocation, which results in the PAX3-FKHR fusion gene. This cell line is commonly used in studies related to alveolar rhabdomyosarcoma, particularly in investigating the molecular mechanisms of tumorigenesis and drug resistance. Rh30 has been utilized in research on the effects of Nutlin-3, a compound that targets the MDM2-p53 interaction, and in studies examining the role of P-glycoprotein in multidrug resistance. Additionally, Rh30 is used to study the expression of genes such as CNR1, PIPOX, and TFABP2, which are differentially expressed in alveolar rhabdomyosarcoma compared to other subtypes. The cell line is also employed in investigations of gene expression profiles and genomic imbalances associated with this cancer type.

Research Applications

Alveolar rhabdomyosarcoma researchMolecular mechanisms of tumorigenesisDrug resistance studiesMDM2-p53 interaction studiesP-glycoprotein function in multidrug resistanceGene expression profilingGenomic imbalance analysis

Key Characteristics

Presence of t(2;13)(q35;q14) translocationPAX3-FKHR fusion geneExpression of CNR1, PIPOX, and TFABP2Use in studies of multidrug resistance
Generated on 6/14/2025

Basic Information

Database IDCVCL_0041
SpeciesHomo sapiens (Human)
Tissue SourceBone marrow[UBERON:UBERON_0002371]

Donor Information

Age17
Age CategoryPediatric
SexMale
Racecaucasian

Disease Information

DiseaseAlveolar rhabdomyosarcoma
LineageSoft Tissue
SubtypeAlveolar Rhabdomyosarcoma
OncoTree CodeARMS

DepMap Information

Source TypeDSMZ
Source IDACH-000833_source

Known Sequence Variations

TypeGene/ProteinDescriptionZygosityNoteSource
Gene fusionFOXO1PAX3-FOXO1, PAX3-FKHR-Not found in original tumorfrom parent cell line Rh30
MutationSimpleRARAp.Pro9Leu (c.26C>T)Heterozygous-from parent cell line Rh30
MutationSimpleTP53p.Tyr205Cys (c.614A>G)Heterozygous-from parent cell line Rh30
MutationSimpleTP53p.Arg273Cys (c.817C>T)Homozygous-PubMed=35933914
MutationSimpleTP53p.Arg280Ser (c.840A>T)Heterozygous-PubMed=31541927, PubMed=23613873, PubMed=18487078, PubMed=15287027

Haplotype Information (STR Profile)

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

Amelogenin
X
CSF1PO
10,11
D10S1248
12
D12S391
20
D13S317
10,11
D16S539
12
D18S51
15,16
D19S433
14,15.2
D1S1656
12,16
D21S11
29,31.2
D22S1045
16,17
D2S1338
17,20
D2S441
11,14
D3S1358
15
D5S818
12,13
D7S820
10
D8S1179
12,15
FGA
22
Penta D
11
Penta E
7
TH01
9,9.3
TPOX
8,11
vWA
17,18
Gene Expression Profile
Gene expression levels and statistical distribution
Loading cohorts...
Full DepMap dataset with combined data across cell lines

Loading gene expression data...

Publications

A landscape of pharmacogenomic interactions in cancer.";

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

Cell 166:740-754(2016).

Surfaceome profiling of cell lines and patient-derived xenografts confirm FGFR4, NCAM1, CD276, and highlight AGRL2, JAM3, and L1CAM as surface targets for rhabdomyosarcoma.

Sala R., Heller M., Rossler J., Bernasconi M.

Int. J. Mol. Sci. 24:2601.1-2601.28(2023).

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).

Heterozygous p.Y955C mutation in DNA polymerase gamma leads to alterations in bioenergetics, complex I subunit expression, and mtDNA replication.

Rahman M.M., Young C.K.J., Goffart S., Pohjoismaki J.L.O., Young M.J.

J. Biol. Chem. 298:102196.1-102196.29(2022).

Assessing alveolar rhabdomyosarcoma cell lines as tumor models by comparison of mRNA expression profiles.

Batchu S., Kellish A.S., Hakim A.A.

Gene 760:145025.1-145025.5(2020).

Quantitative proteomics of the Cancer Cell Line Encyclopedia.";

Sellers W.R., Gygi S.P.

Cell 180:387-402.e16(2020).

Proteomic profiling of rhabdomyosarcoma-derived exosomes yield insights into their functional role in paracrine signaling.

Diab-Assaf M., Saab R., Ghayad S.E.

J. Proteome Res. 18:3567-3579(2019).

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).

Characterization of human cancer cell lines by reverse-phase protein arrays.

Liang H.

Cancer Cell 31:225-239(2017).

Amplification of the gli gene in childhood sarcomas.";

Roberts W.M., Douglass E.C., Peiper S.C., Houghton P.J., Look A.T.

Cancer Res. 49:5407-5413(1989).

A specific chromosomal abnormality in rhabdomyosarcoma.";

Houghton P.J., Houghton J.A., Green A.A.

Cytogenet. Cell Genet. 45:148-155(1987).

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).

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).

Deficiency in rhabdomyosarcomas of a factor required for MyoD activity and myogenesis.

Tapscott S.J., Thayer M.J., Weintraub H.M.

Science 259:1450-1453(1993).

Immunostaining of the p30/32MIC2 antigen and molecular detection of EWS rearrangements for the diagnosis of Ewing's sarcoma and peripheral neuroectodermal tumor.

Lollini P.-L., Picci P., Bertoni F., Baldini N.

Hum. Pathol. 27:408-416(1996).

Bax is an important determinant of chemosensitivity in pediatric tumor cell lines independent of Bcl-2 expression and p53 status.

Harris L.C.

Oncol. Res. 10:235-244(1998).

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).

Potential for treatment of liposarcomas with the MDM2 antagonist Nutlin-3A.

Myklebost O.

Int. J. Cancer 121:199-205(2007).

Global gene expression profiling of PAX-FKHR fusion-positive alveolar and PAX-FKHR fusion-negative embryonal rhabdomyosarcomas.

Olshen A.B., Barr F.G., Ladanyi M.

J. Pathol. 212:143-151(2007).

Reversal of P-glycoprotein-mediated multidrug resistance by the murine double minute 2 antagonist nutlin-3.

Fehse B., Kammerer B., Doerr H.-W., Cinatl J. Jr.

Cancer Res. 69:416-421(2009).

Genomic imbalances in rhabdomyosarcoma cell lines affect expression of genes frequently altered in primary tumors: an approach to identify candidate genes involved in tumor development.

Pritchard-Jones K., Kool M., Shipley J.M.

Genes Chromosomes Cancer 48:455-467(2009).

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).

A genome-wide screen for microdeletions reveals disruption of polarity complex genes in diverse human cancers.

Haber D.A.

Cancer Res. 70:2158-2164(2010).

National Cancer Institute pediatric preclinical testing program: model description for in vitro cytotoxicity testing.

Reynolds C.P.

Pediatr. Blood Cancer 56:239-249(2011).

Inhibition of the Notch-Hey1 axis blocks embryonal rhabdomyosarcoma tumorigenesis.

Belyea B.C., Naini S., Bentley R.C., Linardic C.M.

Clin. Cancer Res. 17:7324-7336(2011).

Oncogene mutation profiling of pediatric solid tumors reveals significant subsets of embryonal rhabdomyosarcoma and neuroblastoma with mutated genes in growth signaling pathways.

Borsu L., Barr F.G., Ladanyi M.

Clin. Cancer Res. 18:748-757(2012).

The Cancer Cell Line Encyclopedia enables predictive modelling of anticancer drug sensitivity.

Morrissey M.P., Sellers W.R., Schlegel R., Garraway L.A.

Nature 483:603-607(2012).

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

Ogawa S.

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

Proof-of-concept rare cancers in drug development: the case for rhabdomyosarcoma.

Sokolowski E., Turina C.B., Kikuchi K., Langenau D.M., Keller C.

Oncogene 33:1877-1889(2014).

Cetuximab promotes anticancer drug toxicity in rhabdomyosarcomas with EGFR amplification in vitro.

Kitagawa Y., Morikawa Y., Kuroda T.

Oncol. Rep. 30:1081-1086(2013).

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

Linardic C.M.

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

A comprehensive transcriptional portrait of human cancer cell lines.

Settleman J., Seshagiri S., Zhang Z.-M.

Nat. Biotechnol. 33:306-312(2015).

Enzastaurin inhibits ABCB1-mediated drug efflux independently of effects on protein kinase C signalling and the cellular p53 status.

Cinatl J. Jr.

Oncotarget 6:17605-17620(2015).

The PAX3-FOXO1 fusion protein present in rhabdomyosarcoma interferes with normal FOXO activity and the TGF-beta pathway.

Schmitt-Ney M., Camussi G.

PLoS ONE 10:E0121474-E0121474(2015).

A resource for cell line authentication, annotation and quality control.

Neve R.M.

Nature 520:307-311(2015).

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).

TCLP: an online cancer cell line catalogue integrating HLA type, predicted neo-epitopes, virus and gene expression.

Loewer M., Sahin U., Castle J.C.

Genome Med. 7:118.1-118.7(2015).