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Mouse Models of Alveolar/Embryonal Rhabdomyosarcoma & Spindle Cell Sarcomas

ABSTRACT: 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.

ORGANISM(S): Mus musculus

SUBMITTER: Hung-I Chen

PROVIDER: E-GEOD-22520 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Evidence for an unanticipated relationship between undifferentiated pleomorphic sarcoma and embryonal rhabdomyosarcoma.

Rubin Brian P BP Nishijo Koichi K Chen Hung-I Harry HI Yi Xiaolan X Schuetze David P DP Pal Ranadip R Prajapati Suresh I SI Abraham Jinu J Arenkiel Benjamin R BR Chen Qing-Rong QR Davis Sean S McCleish Amanda T AT Capecchi Mario R MR Michalek Joel E JE Zarzabal Lee Ann LA Khan Javed J Yu Zhongxin Z Parham David M DM Barr Frederic G FG Meltzer Paul S PS Chen Yidong Y Keller Charles C

Cancer cell 20110201 2

Embryonal rhabdomyosarcoma (eRMS) shows the most myodifferentiation among sarcomas, yet the precise cell of origin remains undefined. Using Ptch1, p53 and/or Rb1 conditional mouse models and controlling prenatal or postnatal myogenic cell of origin, we demonstrate that eRMS and undifferentiated pleomorphic sarcoma (UPS) lie in a continuum, with satellite cells predisposed to giving rise to UPS. Conversely, p53 loss in maturing myoblasts gives rise to eRMS, which have the highest myodifferentiati ...[more]

PMID: 21316601

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