Article
Early signs of neurolipidosis-related behavioural alterations in a murine model of metachromatic leukodystrophy.
Behavioural brain research - 3 Jun 2008
Stroobants Stijn, Leroy Toon, Eckhardt Matthias, Aerts Jean-Marie, Berckmans Daniel, D'Hooge Rudi
Abstract excerpt
Arylsulfatase A (ASA)-deficient mice represent an animal model for the lysosomal storage disorder metachromatic leukodystrophy (MLD). Although the model has been applied in pathophysiological and therapeutic studies, the behavioural phenotype of ASA(-/-) mice is only partially characterized, and the most decisive outcome measures for therapy evaluation only emerge beyond 1 year of age. Presently, ASA(-/-) mice...
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