Molecular mechanism of radiation/NBS1-associated microcephaly

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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.