Neuronal selenoprotein expression is required for interneuron development and prevents seizures and neurodegeneration.

ABSTRACT: Cerebral selenium (Se) deficiency is associated with neurological phenotypes including seizures and ataxia. We wanted to define whether neurons require selenoprotein expression and which selenoproteins are most important, and explore the possible pathomechanism. Therefore, we abrogated the expression of all selenoproteins in neurons by genetic inactivation of the tRNA[Ser](Sec) gene. Cerebral expression of selenoproteins was significantly diminished in the mutants, and histological analysis revealed progressive neurodegeneration. Developing interneurons failed to specifically express parvalbumin (PV) in the mutants. Electrophysiological recordings, before overt cell death, showed normal excitatory transmission, but revealed spontaneous epileptiform activity consistent with seizures in the

SUBMITTER: Wirth EK 

PROVIDER: S-EPMC2830140 | biostudies-literature | 2010 Mar

REPOSITORIES: biostudies-literature