The Twitcher mouse: an enzymatically authentic model of human globoid cell leukodystrophy (Krabbe disease).

The Twitcher is a new neurological mutant of mouse which shows clinical and histopathological features similar to those of human and canine globoid cell leukodystrophy (Krabbe disease). Brain and liver tissues of affected mice showed a profound deficiency of galactosylceramidase and lactosylceramidase I activities. A distinct group with intermediate enzyme… CONTINUE READING