Whole Transcriptome Screening Reveals Myelination Deficits in Dysplastic Human Temporal Neocortex

摘要: Focal cortical dysplasias (FCDs) are local malformations of the human neocortex with strong epileptogenic potential. To investigate underlying pathomechanisms, we performed a whole transcriptome screening to compare gene expression pattern dysplastic versus nondysplastic temporal neocortex. Tissue obtained from FCD IIIa cases (mean age 20.5 years) who had undergone surgical treatment, due intractable epilepsy, was compared specimens 19.9 by means Affymetrix arrays covering 28 869 genes. We found 211 differentially expressed genes (DEX) among which mainly important for oligodendrocyte differentiation and myelination were downregulated in IIIa. These findings confirmed as functionally Database Annotation, Visualization, Integrated Discovery (DAVID) analysis. The reduced myelin-associated transcripts Ia, IIa, real-time RT-qPCR. In addition, that density myelin basic protein mRNA-expressing oligodendrocytes 2',3'-cyclic nucleotide 3'-phosphodiesterase-positive fibers significantly cortex. Moreover, high-resolution confocal imaging 3D reconstruction revealed fiber network severely disorganized neocortex, indicating disturbance sheath formation maintenance FCD.