Article
Expression of kinase-deficient MEK2 ameliorates Pelizaeus-Merzbacher disease phenotypes in mice.
Biochemical and biophysical research communications - 22 Oct 2020
Miyamoto Yuki, Tanaka Marina, Ito Hisanaka, Ooizumi Hiroaki, Ohbuchi Katsuya, Mizoguchi Kazushige, Torii Tomohiro, Yamauchi Junji
Abstract excerpt
Pelizaeus-Merzbacher disease (PMD) is characterized as a congenital hypomyelinating disorder in oligodendrocytes, myelin-forming glial cells in the central nervous system (CNS). The responsible gene of PMD is plp1, whose multiplication, deletion, or mutation is associated with PMD. We previously reported that primary oligodendrocytes overexpressing proteolipid protein 1 (PLP1) do not have the ability to...
Topics
- Animals
- Brain
- Disease Models, Animal
- Female
- Gene Expression Regulation, Enzymologic
- Genes, Dominant
- MAP Kinase Kinase 2
- MAP Kinase Signaling System
- Male
- Mice, Transgenic