Table 1 Mouse models of human sarcomas
From: Mouse models of sarcomas: critical tools in our understanding of the pathobiology
Tumor type | Gene alteration(s) | Agent used | Significance | Proposed karyotype | Reference |
|---|---|---|---|---|---|
Soft Tissue Sarcoma | ND | Rous sarcoma virus | Viral infection influences sarcomagenesis | Unknown | [5] |
Osteosarcoma | ND | Radiation | Radiation influences 7 osteosarcoma formation in rabbits | Unknown | [7] |
Sporadic/ Varied | p53−/− | None | Loss of p53 results in sarcoma formation | Complex | |
Sporadic/ Varied | p53R172H mutation | None | Mutations in p53 results in sarcoma formation | Complex | |
Sporadic/ 31 Varied | Mdm2SNP309 w or w/o p53R172Hmutation | None | Mutations in the p53 pathway result in sarcoma formation | Complex | [31] |
Poorly Differentiated Sarcoma | Ews-Fli1Tg w or w/o p53 deletion | Prx-Cre | Ews-Fli1Tgmice fail to induce sarcomagenesis in the absence of p53 loss | Translocation but complex with p53/Rb loss | [44] |
Poorly Differentiated Sarcoma/ Osteosarcoma | Ews-Fli1Tg w or w/o Rb and p53 deletions | Prx-Cre | Ews-Fli1Tg;Rb−/− mice fail to induce sarcomagenesis but reduce time of onset in the absence of p53 | Translocation but complex with p53/Rb loss | [45] |
Osteosarcoma | Tax Tg ;p19Arf −/− | None | Expression of Tax in the absence of p19Arf results in osteosarcoma formation | Complex | [46] |
Osteosarcoma | Rb and p53 deletions | Osterix-Cre | Deletion of p53 and Rb cooperate in the bone leading to osteosarcomagenesis | Complex | [47] |
Osteosarcoma/ Leiomyosarcoma | Rb +/- and p107 deletions | None | Rb haploinsufficiency coupled with p107 deletion results in low penetrant Sarcoma formation | Complex | [48] |
Developmental defects | Pax-3-Fkhr fusion | None | Pax3-Fkhr fusion product fails to produce sarcomas when expressed from Pax3 promoter | Translocation | [49] |
Rhabdomyosarcomas | Pax3-Fkhr transgene w/ and w/o Rb and p53 deletion | Myf6-Cre | Expression of the Pax3-Fkhr transgene requires loss of p53 and Rb for rhabdomyosarcoma formation | Translocation but complex with p53/Rb loss | |
Rhabdomyosarcomas/ Various sarcomas | Ptch1 heterozygosity w/ and w/o Rb and p53 deletion | Myf6-Cre, Myf-5Cre, or Pax7-CreER | Loss of p53 and Rb in conjunction with Ptch1 haploinsufficiency results in rhabdomyosarcomagenesis | Complex | [52] |
Undifferentiated Pleomorphic sarcomas | Mutant K-ras expression and p53 loss | Adenoviral Cre | MutantK-ras expression and p53 loss cooperate in the development of undifferentiated pleomorphic sarcomas | Complex | |
Pleomorphic Rhabdomyosarcomas | Mutant K-ras expression and p53 loss | Electroporation of Cre into the muscle | MutantK-ras expression and p53 loss cooperate in the development of undifferentiated pleomorphic sarcomas | Complex | [55] |
Rhabdomyosarcomas | Mutant K-ras expression and p53 loss or mutation of p53 | Ah-Cre | Expression of mutant p53 facilitates a more rapid development of rhabdomyosarcomas than loss of p53 in the mutant K-ras background | Complex | [56] |