التفاصيل البيبلوغرافية
| العنوان: |
Evidence for mTOR pathway activation in a spectrum of epilepsy-associated pathologies |
| المؤلفون: |
Liu, Joan, Reeves, Cheryl, Michalak, Zuzanna, Coppola, Antonietta, Diehl, Beate, Sisodiya, Sanjay M, Thom, Maria |
| بيانات النشر: |
BioMed Central Ltd. |
| سنة النشر: |
2014 |
| المجموعة: |
BioMed Central |
| مصطلحات موضوعية: |
mTOR pathway, Epilepsy, Neuronal dysplasia, Hippocampal sclerosis, Rasmussen’s’ encephalitis |
| الوصف: |
Introduction Activation of the mTOR pathway has been linked to the cytopathology and epileptogenicity of malformations, specifically Focal Cortical Dysplasia (FCD) and Tuberous Sclerosis (TSC). Experimental and clinical trials have shown than mTOR inhibitors have anti-epileptogenic effects in TS. Dysmorphic neurones and balloon cells are hallmarks of FCDIIb and TSC, but similar cells are also occasionally observed in other acquired epileptogenic pathologies, including hippocampal sclerosis (HS) and Rasmussen’s encephalitis (RE). Our aim was to explore mTOR pathway activation in a range of epilepsy-associated pathologies and in lesion-negative cases. Results 50 epilepsy surgical pathologies were selected including HS ILAE type 1 with (5) and without dysmorphic neurones (4), FCDIIa (1), FCDIIb (5), FCDIIIa (5), FCDIIIb (3), FCDIIId (3), RE (5) and cortex adjacent to cavernoma (1). We also included pathology-negative epilepsy cases; temporal cortex (7), frontal cortex (2), paired frontal cortical samples with different ictal activity according to intracranial EEG recordings (4), cortex with acute injuries from electrode tracks (5) and additionally non-epilepsy surgical controls (3). Immunohistochemistry for phospho-S6 (pS6) ser240/244 and ser235/236 and double-labelling for Iba1, neurofilament, GFAP, GFAPdelta, doublecortin, and nestin were performed. Predominant neuronal labelling was observed with pS6 ser240/244 and glial labelling with pS6 ser235/236 in all pathology types but with evidence for co-expression in a proportion of cells in all pathologies. Intense labelling of dysmorphic neurones and balloon cells was observed in FCDIIb, but dysmorphic neurones were also labelled in RE and HS. There was no difference in pS6 labelling in paired samples according to ictal activity. Double-labelling immunofluorescent studies further demonstrated the co-localisation of pS6 with nestin, doublecortin, GFAPdelta in populations of small, immature neuroglial cells in a range of epilepsy pathologies. Conclusions ... |
| نوع الوثيقة: |
report |
| اللغة: |
English |
| العلاقة: |
http://www.actaneurocomms.org/content/2/1/71Test |
| الإتاحة: |
http://www.actaneurocomms.org/content/2/1/71Test |
| حقوق: |
Copyright 2014 Liu et al.; licensee BioMed Central Ltd. |
| رقم الانضمام: |
edsbas.A0AF3852 |
| قاعدة البيانات: |
BASE |
