Teriflunomide attenuates neuroinflammation-related neural damage in mice carrying human PLP1 mutations.

ABSTRACT:

Background

Genetically caused neurological disorders of the central nervous system (CNS) are mostly characterized by poor or even fatal clinical outcome and few or no causative treatments are available. Often, these disorders are associated with low-grade, disease-promoting inflammation, another feature shared by progressive forms of multiple sclerosis (PMS). We previously generated two mouse lines carrying distinct mutations in the oligodendrocytic PLP1 gene that have initially been identified in patients diagnosed with MS. These mutations cause a loss of PLP function leading to a histopathological and clinical phenotype common to both PMS and genetic CNS disorders, like hereditary spastic paraplegias. Importantly, neuroinflammation promotes disease progression in these models, su

SUBMITTER: Groh J 

PROVIDER: S-EPMC6031103 | biostudies-literature | 2018 Jul

REPOSITORIES: biostudies-literature