Hypervascularization in mTOR-dependent focal and global cortical malformations displays differential rapamycin sensitivity.

ABSTRACT:

Objectives

Patients with mammalian target of rapamycin (mTOR)-dependent malformations of cortical development (MCDs) associated with seizures display hyperperfusion and increased vessel density of the dysmorphic cortical tissue. Some studies have suggested that the vascular defect occurred independently of seizures. Here, we further examined whether hypervascularization occurs in animal models of global and focal MCD with and without seizures, and whether it is sensitive to the mTOR blocker, rapamycin, that is approved for epilepsy treatment in tuberous sclerosis complex.

Methods

We used two experimental models of mTOR-dependent MCD consisting of conditional transgenic mice containing Tsc1^null cells in the forebrain generating a global malformation associated with

SUBMITTER: Zhang L 

PROVIDER: S-EPMC6558978 | biostudies-literature | 2019 Jun

REPOSITORIES: biostudies-literature