%0 Journal Article
%T Bone Marrow Transplantation Alters the Tremor Phenotype in the Murine Model of Globoid-Cell Leukodystrophy
%A Adarsh S. Reddy
%A David F. Wozniak
%A Nuri B. Farber
%A Joshua T. Dearborn
%A Stephen C. Fowler
%A Mark S. Sands
%J Journal of Clinical Medicine
%D 2012
%I MDPI AG
%R 10.3390/jcm1010001
%X Tremor is a prominent phenotype of the twitcher mouse, an authentic genetic model of Globoid-Cell Leukodystrophy (GLD, Krabbe¡¯s disease). In the current study, the tremor was quantified using a force-plate actometer designed to accommodate low-weight mice. The actometer records the force oscillations caused by a mouse¡¯s movements, and the rhythmic structure of the force variations can be revealed. Results showed that twitcher mice had significantly increased power across a broad band of higher frequencies compared to wildtype mice. Bone marrow transplantation (BMT), the only available therapy for GLD, worsened the tremor in the twitcher mice and induced a measureable alteration of movement phenotype in the wildtype mice. These data highlight the damaging effects of conditioning radiation and BMT in the neonatal period. The behavioral methodology used herein provides a quantitative approach for assessing the efficacy of potential therapeutic interventions for Krabbe¡¯s disease.
%K bone marrow transplantation
%K lysosomal storage disease
%K tremor
%K galactosylceramidase
%K globoid-cell leukodystrophy
%U http://www.mdpi.com/2077-0383/1/1/1