TY - JOUR
T1 - Mammalian target of rapamycin (mTOR) activation in focal cortical dysplasia and related focal cortical malformations
AU - Wong, Michael
PY - 2013/6
Y1 - 2013/6
N2 - Focal cortical dysplasia (FCD) and other localized malformations of cortical development represent common causes of intractable pediatric epilepsy. Insights into the cellular and molecular pathogenesis of focal cortical malformations may reveal information about associated mechanisms of epileptogenesis and suggest new therapies for seizures caused by these developmental lesions. In animal models and human studies of FCD and the related disease of Tuberous Sclerosis Complex (TSC), the mammalian target of rapamycin (mTOR) pathway has been implicated in mediating cellular and molecular changes leading to the formation of the cortical malformations and the expression of epilepsy. The use of mTOR inhibitors may represent a rational therapeutic strategy for treating or even preventing epilepsy due to FCD and TSC.
AB - Focal cortical dysplasia (FCD) and other localized malformations of cortical development represent common causes of intractable pediatric epilepsy. Insights into the cellular and molecular pathogenesis of focal cortical malformations may reveal information about associated mechanisms of epileptogenesis and suggest new therapies for seizures caused by these developmental lesions. In animal models and human studies of FCD and the related disease of Tuberous Sclerosis Complex (TSC), the mammalian target of rapamycin (mTOR) pathway has been implicated in mediating cellular and molecular changes leading to the formation of the cortical malformations and the expression of epilepsy. The use of mTOR inhibitors may represent a rational therapeutic strategy for treating or even preventing epilepsy due to FCD and TSC.
KW - Epilepsy
KW - Malformation of cortical development
KW - Seizure
KW - Tuberous sclerosis complex
UR - http://www.scopus.com/inward/record.url?scp=84877589482&partnerID=8YFLogxK
U2 - 10.1016/j.expneurol.2011.10.002
DO - 10.1016/j.expneurol.2011.10.002
M3 - Review article
C2 - 22015915
AN - SCOPUS:84877589482
SN - 0014-4886
VL - 244
SP - 22
EP - 26
JO - Experimental Neurology
JF - Experimental Neurology
ER -