Spatiotemporal Gradient of Cortical Neuron Death Contributes to Microcephaly in Knock-In Mouse Model of Ligase 4 Syndrome

Cells of the developing central nervous system are particularly susceptible to formation of double-stranded DNA breaks (DSBs) arising from physiological and/or environmental insults. Therefore, efficient repair of DSBs is especially vital for maintaining cellular health and proper functioning in the...

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Veröffentlicht in:	The American journal of pathology 2019-12, Vol.189 (12), p.2440-2449
Hauptverfasser:	Lun, Melody P., Shannon, Morgan L., Keles, Sevgi, Reisli, Ismail, Luche, Nicole, Ryan, Douglas, Capuder, Kelly, Notarangelo, Luigi D., Lehtinen, Maria K.
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Apoptosis Cerebral Cortex - metabolism Cerebral Cortex - pathology Craniofacial Abnormalities - complications Disease Models, Animal DNA Breaks, Double-Stranded DNA Ligase ATP - genetics DNA Ligase ATP - metabolism Female Gene Knock-In Techniques Growth Disorders - complications Immunologic Deficiency Syndromes - complications Male Mice Microcephaly - etiology Microcephaly - pathology Mutation Neurons - metabolism Neurons - pathology Spatio-Temporal Analysis
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