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FCDs are classified into different subtypes including FCD IIa and IIb, characterized by a blurred gray\u2010white matter boundary or a transmantle sign indicating abnormal white matter myelination. Recently, we have shown that myelination is also compromised in the gray matter of FCD IIa of the temporal lobe. Since myelination is key for brain function, which is imbalanced in epilepsy, in the current study, we investigated myelination in the gray matter of FCD IIa and IIb from the frontal lobe on the morphological, ultrastructural, and transcriptional level. We found that FCD IIa presents with an ordinary radial myelin fiber pattern, but with a reduced thickness of myelin sheaths of 500\u20131000\u2009nm thick axons in comparison to FCD IIb and with an attenuation of the myelin synthesis machinery. In contrast, FCD IIb showed an irregular and disorganized myelination pattern covering an enlarged area in comparison to FCD IIa and controls and with increased numbers of myelinating oligodendrocytes (OLs). FCD IIb had significantly thicker myelin sheaths of large caliber axons (above 1000\u2009nm) when compared to FCD IIa. Accordingly, FCD IIb showed a significant up\u2010regulation of myelin\u2010associated mRNAs in comparison to FCD IIa and enhanced binding capacities of the transcription factor MYRF to target sites in myelin\u2010associated genes. These data indicate that FCD IIa and IIb are characterized by a differential dysregulation of myelination in the gray matter of the frontal lobe.<\/jats:p>","DOI":"10.1002\/glia.24662","type":"journal-article","created":{"date-parts":[[2024,12,25]],"date-time":"2024-12-25T00:54:36Z","timestamp":1735088076000},"page":"928-947","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Dysregulation of Myelination in Focal Cortical Dysplasia Type\n                    <scp>II<\/scp>\n                    of the Human Frontal Lobe"],"prefix":"10.1002","volume":"73","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2636-3873","authenticated-orcid":false,"given":"Catharina","family":"Donkels","sequence":"first","affiliation":[{"name":"Faculty of Medicine, Experimental Epilepsy Research, Department of Neurosurgery Medical Center \u2010 University of Freiburg  Freiburg 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