Figure 7
From: A distinct response to endogenous DNA damage in the development of Nbs1-deficient cortical neurons

Working model for Nbs1 function and regulation in neocortex. Endogenous DNA damage during rapid development of neocortical neurons activates ATM-Chk2 and ATR-Chk1 signaling. However, defective Nbs1 impairs ATR-Chk1 pathway rather than ATM-Chk2 signaling. ATM-Chk2 signaling in Nbs1-deficient neocortex activates p53-Hzf pathway and induces defective proliferation rather than apoptosis of neuroprogenitors, leading to microcephaly.