Fig. 7

Fanconi Anemia Pathway. Endogenous and exogenous agent cause interstrand crosslink lesions (ICL). When an ICL occurs, the replication fork is stalled and the FA pathway is activated with the recruitment of the FA core complex (FANCA, B, C, E, F, G, L, and M). FANCL ubiqiuitinates the FANCD2-FANCI (ID2) complex that guides the nucleolytic incisions and translesion synthesis repair events by “unhooking” the ICL. This allows the homologous recombination repair through FANCD1/BRCA2, FANCJ/BRIP1, FANCN/PALB2, FANCO/RAD51C, and FANCS/BRCA1. Yellow stars highlight FA/HR repair molecules that have been found genetically inactivated in FA patients with medulloblastoma. BCCIP is a BRCA2 interacting protein which is able to induce medulloblastoma growth when genetically loss in concomitance with p53 deletion (See text). Readapted from [52]