Figure 3.

Three putative mechanisms of chromosomal translocation initiating events in ARMS development. (1) ARMS forms as a result of de novo translocation leading to the formation of PAX3-FKHR oncoprotein that convert cells into a permissible state for acquiring additional mutations. (2) ARMS forms from cells with existing genetic mutation that predisposes to chromosomal translocation. (3)De novo translocation leading to the co-expression of PAX3-FKHR and FKHR-PAX3 is sufficient to initiate the ARMS phenotype. The non-translocation mutations shown in the first two models may be variable, contributing to the heterogeneity of ARMS.