ID   JN-DSRCT-1
AC   CVCL_9W68
SY   JN-DSRCT
DR   cancercelllines; CVCL_9W68
DR   Cosmic; 1718818
DR   Wikidata; Q54899015
RX   PubMed=12218078;
RX   PubMed=15782132;
RX   PubMed=22142829;
RX   PubMed=34413129;
RX   PubMed=34841430;
CC   Population: Japanese.
CC   Doubling time: ~38 hours (PubMed=12218078); 22 hours (PubMed=34841430).
CC   Sequence variation: Gene fusion; HGNC; 3508; EWSR1 + HGNC; 12796; WT1; Name(s)=EWSR1-WT1, EWS-WT1; Note=EWSR1 Ex10-WT1 Ex8 fusion (PubMed=12218078; PubMed=34841430).
CC   Sequence variation: Mutation; HGNC; 2348; CREBBP; Simple; p.Arg1443Alafs*11; Zygosity=Unspecified (PubMed=34841430).
CC   Sequence variation: Mutation; HGNC; 4172; GATA3; Simple; p.Arg367Ter (c.1099C>T); ClinVar=VCV000016626; Zygosity=Unspecified (PubMed=34841430).
CC   Sequence variation: Mutation; HGNC; 9884; RB1; Simple; p.Arg621Ser (c.1861C>A); ClinVar=VCV000135120; Zygosity=Unspecified (PubMed=34841430).
CC   Derived from site: Metastatic; Pleural effusion; UBERON=UBERON_0000175.
DI   NCIt; C27372; Childhood desmoplastic small round cell tumor
DI   ORDO; Orphanet_83469; Desmoplastic small round cell tumor
OX   NCBI_TaxID=9606; ! Homo sapiens (Human)
SX   Male
AG   7Y
CA   Cancer cell line
DT   Created: 23-02-16; Last updated: 05-10-23; Version: 13
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RX   PubMed=12218078; DOI=10.1097/01.LAB.0000028059.92642.03;
RA   Nishio J., Iwasaki H., Ishiguro M., Ohjimi Y., Fujita C., Yanai F.,
RA   Nibu K., Mitsudome A., Kaneko Y., Kikuchi M.;
RT   "Establishment and characterization of a novel human desmoplastic
RT   small round cell tumor cell line, JN-DSRCT-1.";
RL   Lab. Invest. 82:1175-1182(2002).
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RX   PubMed=15782132; DOI=10.1038/sj.onc.1208471;
RA   Tirado O.M., Mateo-Lozano S., Notario V.;
RT   "Rapamycin induces apoptosis of JN-DSRCT-1 cells by increasing the
RT   Bax:Bcl-xL ratio through concurrent mechanisms dependent and independent
RT   of its mTOR inhibitory activity.";
RL   Oncogene 24:3348-3357(2005).
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RX   PubMed=22142829; DOI=10.1158/1078-0432.CCR-11-2056;
RA   Shukla N., Ameur N., Yilmaz I., Nafa K., Lau C.-Y., Marchetti A.,
RA   Borsu L., Barr F.G., Ladanyi M.;
RT   "Oncogene mutation profiling of pediatric solid tumors reveals
RT   significant subsets of embryonal rhabdomyosarcoma and neuroblastoma
RT   with mutated genes in growth signaling pathways.";
RL   Clin. Cancer Res. 18:748-757(2012).
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RX   PubMed=34413129; DOI=10.1158/1535-7163.MCT-21-0089;
RA   Gartrell J., Mellado-Largarde M., Clay M.R., Bahrami A., Sahr N.A.,
RA   Sykes A., Blankenship K., Hoffmann L., Xie J., Cho H.P., Twarog N.,
RA   Connelly M., Yan K.-K., Yu J.-Y., Porter S.N., Pruett-Miller S.M.,
RA   Neale G., Tinkle C.L., Federico S.M., Stewart E.A., Shelat A.A.;
RT   "SLFN11 is widely expressed in pediatric sarcoma and induces variable
RT   sensitization to replicative stress caused by DNA-damaging agents.";
RL   Mol. Cancer Ther. 20:2151-2165(2021).
//
RX   PubMed=34841430; DOI=10.1242/dmm.047621;
RA   Smith R.S., Odintsov I., Liu Z.-B., Lui A.J.-W., Hayashi T., Vojnic M.,
RA   Suehara Y., Delasos L., Mattar M.S., Hmeljak J., Ramirez H.A.,
RA   Shaw M., Bui G., Hartono A.B., Gladstone E., Kunte S., Magnan H.,
RA   Khodos I., de Stanchina E., La Quaglia M.P., Yao J.-J., Lae M.,
RA   Lee S.B., Spraggon L., Pratilas C.A., Ladanyi M., Somwar R.;
RT   "Novel patient-derived models of DSRCT enable validation of ERBB
RT   signaling as a potential therapeutic vulnerability.";
RL   Dis. Model. Mech. 15:dmm047621.1-dmm047621.15(2022).
//