Congenitally hypomyelinated shiverer mice fail to generate compact myelin and die by 18-21 selleckchem ABT-199 weeks of age. Employing multifocal anterior and posterior fossa delivery of sorted fetal human glial progenitor cells into neonatal shiverer x rag2(-/-) mice, we attained total neuraxis myelination in the engrafted hosts, which in a significant fraction of circumstances rescued this otherwise MAO lethal phenotype. The transplanted mice exhibited greatly prolonged survival with progressive resolution of their neurological deficits. Substantial myelination in several areas was accompanied through the acquisition of regular nodes of Ranvier and transcallosal conduction velocities, ultrastructurally normal and comprehensive myelination of most axons, along with a restoration of the considerably regular neurological phenotype. Notably, the resultant mice have been cerebral chimeras, with murine gray matter but a predominantly human white matter glial composition. These data show that the neonatal transplantation of human glial progenitor cells can efficiently treat disorders of congenital and perinatal hypomyelination.