Blood-brain barrier permeability to horseradish peroxidase in twitcher and cuprizone-intoxicated mice.

The status of blood-brain barrier (BBB) permeability was investigated in the twitcher, an authentic murine model of globoid cell leukodystrophy (GLD, Krabbe disease) and cuprizone-intoxicated mice. Although extensive demyelination was noted in the CNS of both mice and additionally, macrophage infiltration was pronounced in the twitcher, BBB remained intact to horseradish peroxidase. Thus, the change of microenvironment caused by demyelination is not necessarily a responsible factor for increased BBB permeability in the inflammatory demyelinating conditions.