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.