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J. Clin. Med. 2012, 1(1), 1-14; doi:10.3390/jcm1010001
Article

Bone Marrow Transplantation Alters the Tremor Phenotype in the Murine Model of Globoid-Cell Leukodystrophy

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1 Department of Internal Medicine, Campus box 8007, 660 South Euclid Avenue, Washington University School of Medicine, St. Louis, MO 63110, USA 2 Department of Psychiatry, Campus Box 8134, 660 S. Euclid Avenue, Washington University School of Medicine, St. Louis, MO 63110, USA 3 Department of Pharmacology and Toxicology, University of Kansas, Lawrence, KS 66045, USA
* Authors to whom correspondence should be addressed.
Received: 9 December 2011 / Revised: 7 January 2012 / Accepted: 11 January 2012 / Published: 19 January 2012
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Abstract

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.
Keywords: bone marrow transplantation; lysosomal storage disease; tremor; galactosylceramidase; globoid-cell leukodystrophy bone marrow transplantation; lysosomal storage disease; tremor; galactosylceramidase; globoid-cell leukodystrophy
This is an open access article distributed under the Creative Commons Attribution License (CC BY 3.0).

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Reddy, A.S.; Wozniak, D.F.; Farber, N.B.; Dearborn, J.T.; Fowler, S.C.; Sands, M.S. Bone Marrow Transplantation Alters the Tremor Phenotype in the Murine Model of Globoid-Cell Leukodystrophy. J. Clin. Med. 2012, 1, 1-14.

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