Figure 4

Retinoblastoma and FA/BRCA pathway transformation model. First and second hits in retinoblastoma are the inactivation of the RB1 alleles. Gain of E2F3 copy numbers aids to the upregulation of specific FA genes. In later stage inactivation of the FA/BRCA-pathway may be accomplished via low levels of the FANCF DNA/RNA, resulting in melphalan sensitivity.