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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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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 which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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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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