Cortical interneuron development is affected in 4H leukodystrophy 55 2 Figure 8. 4H neurons show increased expression of parvalbumin interneuron marker ERBB4. QPCR analysis for neuronal and GABAergic markers on day 30 and day 56 control and 4H neurons. (A) NEUN expression, which is decreased in day 30 4H neurons; therefore B-H are corrected for both NEUN and EIF4G2 expression. (B) Expression of DLX2 decreased over time in control neurons, which is less pronounced in 4H cells. (C-H) qPCR analysis of specific interneuron subtypes: (C) RELN, an early marker for NDNF interneurons, (D) NDNF, (E) NRG1, an early marker for ID2 interneurons, (F) CNTNAP, an early marker for VIP interneurons, (G) PDE1A, an early marker for SST interneurons and (H) ERBB4, an early marker for PV interneurons. ERBB4 expression was significantly increased in 4H neurons at day 30 and day 56 (H). AU = arbitrary units, * P < 0.05
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