Molecular mechanism of radiation/NBS1-associated microcephaly

放射性小头畸形的发生率是放射性致癌的10倍。我们向她展示了辐射诱导基底神经前体细胞凋亡，并发展成辐射性小头畸形。同时，在顶端神经前体细胞中观察到凋亡，所述顶端神经前体细胞通过中心体附着到顶端层的细胞增殖产生基底神经前体细胞。这些证据表明，增殖是至关重要的发展辐射apososis，因为缺陷，在维持中心体诱导小头畸形的患者Nijmege断裂综合征。

The incidence of radiation microcephaly is 10-fold higher than that of radiation carcinogenesis. We showed her that radiation induces the apoptosis in basal neuro-progenitor cells, and develops radiation microcephaly. Simultaneously, apoptosis was observed in apical neuro-progenitor cells, which produce basal neuro-progenitor cells by cell proliferation through attachment of centrosomes to apical layers. These evidences suggest that proliferation is critical to develop radiation apososis, because defect in maintaining centrosomes induces the microcephaly in a patient with Nijmege breakage syndrome.