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Bone Marrow Transplantation Alters the Tremor Phenotype in the Murine Model of Globoid-Cell Leukodystrophy
Department of Internal Medicine, Campus box 8007, 660 South Euclid Avenue, Washington University School of Medicine, St. Louis, MO 63110, USA
Department of Psychiatry, Campus Box 8134, 660 S. Euclid Avenue, Washington University School of Medicine, St. Louis, MO 63110, USA
Department of Pharmacology and Toxicology, University of Kansas, Lawrence, KS 66045, USA
* Authors to whom correspondence should be addressed.
Received: 9 December 2011; in revised form: 7 January 2012 / Accepted: 11 January 2012 / Published: 19 January 2012
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
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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.
Reddy AS, Wozniak DF, Farber NB, Dearborn JT, Fowler SC, Sands MS. Bone Marrow Transplantation Alters the Tremor Phenotype in the Murine Model of Globoid-Cell Leukodystrophy. Journal of Clinical Medicine. 2012; 1(1):1-14.
Reddy, Adarsh S.; Wozniak, David F.; Farber, Nuri B.; Dearborn, Joshua T.; Fowler, Stephen C.; Sands, Mark S. 2012. "Bone Marrow Transplantation Alters the Tremor Phenotype in the Murine Model of Globoid-Cell Leukodystrophy." J. Clin. Med. 1, no. 1: 1-14.