dc.contributor.author | Reddy, Adarsh S. | |
dc.contributor.author | Wozniak, David F. | |
dc.contributor.author | Farber, Nuri B. | |
dc.contributor.author | Dearborn, Joshua T. | |
dc.contributor.author | Fowler, Stephen C. | |
dc.contributor.author | Sands, Mark S. | |
dc.date.accessioned | 2015-05-11T15:18:17Z | |
dc.date.available | 2015-05-11T15:18:17Z | |
dc.date.issued | 2012-01-19 | |
dc.identifier.citation | Reddy, 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/jcm1010001 | en_US |
dc.identifier.uri | http://hdl.handle.net/1808/17677 | |
dc.description | This is the publisher's version, also available electronically from "http://www.mdpi.com". | en_US |
dc.description.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. | en_US |
dc.publisher | MDPI | en_US |
dc.subject | bone marrow transplantation | en_US |
dc.subject | lysosomal storage disease | en_US |
dc.subject | tremor | en_US |
dc.subject | galactosylceramidase | en_US |
dc.subject | globoid-cell leukodystrophy | en_US |
dc.title | Bone Marrow Transplantation Alters the Tremor Phenotype in the Murine Model of Globoid-Cell Leukodystrophy | en_US |
dc.type | Article | |
kusw.kuauthor | Fowler, Stephen C. | |
kusw.kudepartment | Pharmacology & Toxicology | en_US |
dc.identifier.doi | 10.3390/jcm1010001 | |
kusw.oaversion | Scholarly/refereed, publisher version | |
kusw.oapolicy | This item meets KU Open Access policy criteria. | |
dc.rights.accessrights | openAccess | |