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Patients lacking certain components of the DNA non-homologous end-joining (NHEJ) pathway (eg.DNA ligase Ⅳ) display microcephaly and development delay at birth.A-T patients have normal head circumference at birth but develop progressive ataxia.To explore the basis underlying microcephaly observed in LIG4 Syndrome patients, we have examined DNA double strand break (DSB) formation and the response to DSBs in a mouse model for LIG4 Syndrome.