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Dev Neurosci ; 42(5-6): 230-236, 2020.
Article in English | MEDLINE | ID: mdl-33706310

ABSTRACT

The tuberous sclerosis complex (TSC), focal cortical dysplasia IIB (FCD IIB), and hemimegalencephaly (HME) exhibit similar molecular features that are dependent on the hyperactivation of the mTOR pathway. They are all associated with refractory epilepsy and the need for surgical resection with varying outcomes. The phosphorylated protein S6 (pS6) is a downstream target of mTOR, whose increased expression might indicate mTOR hyperactivation, but which is also present when there is no alteration in the pathway (such as in FCD type I). We have performed immunohistochemical marking and quantification of pS6 in resected brain specimens of 26 patients clinically and histologically diagnosed with TSC, FCD IIB, or HME and compared this data to a control group of 25 patients, to measure the extent of pS6 positivity and its correlation with clinical aspects. Our results suggest that pS6 may serve as a reliable biomarker in epilepsy and that a greater percentage of pS6 marking can relate to more severe forms of mTOR-dependent brain anomalies.


Subject(s)
Biomarkers/metabolism , Drug Resistant Epilepsy/metabolism , Ribosomal Protein S6/metabolism , Adolescent , Child , Child, Preschool , Drug Resistant Epilepsy/etiology , Drug Resistant Epilepsy/surgery , Epilepsy/complications , Epilepsy/metabolism , Epilepsy/surgery , Female , Hemimegalencephaly/complications , Hemimegalencephaly/metabolism , Hemimegalencephaly/surgery , Humans , Infant , Male , Malformations of Cortical Development, Group I/complications , Malformations of Cortical Development, Group I/metabolism , Malformations of Cortical Development, Group I/surgery , Phosphorylation , Tuberous Sclerosis/complications , Tuberous Sclerosis/metabolism , Tuberous Sclerosis/surgery
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