Glial progenitor cell-based treatment of the childhood leukodystrophies.

ABSTRACT: The childhood leukodystrophies comprise a group of hereditary disorders characterized by the absence, malformation or destruction of myelin. These disorders share common clinical, radiological and pathological features, despite their diverse molecular and genetic etiologies. Oligodendrocytes and astrocytes are the major affected cell populations, and are either structurally impaired or metabolically compromised through cell-intrinsic pathology, or are the victims of mis-accumulated toxic byproducts of metabolic derangement. In either case, glial cell replacement using implanted tissue or pluripotent stem cell-derived human neural or glial progenitor cells may comprise a promising strategy for both structural remyelination and metabolic rescue. A broad variety of pediatric white matter diso

SUBMITTER: Osorio MJ 

PROVIDER: S-EPMC5340082 | biostudies-literature | 2016 Sep

REPOSITORIES: biostudies-literature