Reddy, Adarsh S.Wozniak, David F.Farber, Nuri B.Dearborn, Joshua T.Fowler, Stephen C.Sands, Mark S.2015-05-112015-05-112012-01-19Reddy, A. S., Wozniak, D. F., Farber, N. B., Dearborn, J. T., Fowler, S. C. & Sands, M. S. (2012) Bone Marrow Transplantation Alters the Tremor Phenotype in the Murine Model of Globoid-Cell Leukodystrophy. Journal of Clinical Medicine, 1(1), 1-14. http://www.dx.doi.org/10.3390/jcm1010001https://hdl.handle.net/1808/17677This is the publisher's version, also available electronically from "http://www.mdpi.com".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.bone marrow transplantationlysosomal storage diseasetremorgalactosylceramidasegloboid-cell leukodystrophyArticle10.3390/jcm1010001openAccess