Working with mice, University of Wisconsin-Madison researchers have developed the basis for a therapeutic strategy that could provide hope for children afflicted with Krabbe’s disease, a fatal nervous system disorder.
Writing this week (Dec. 12, 2005) in the Proceedings of the National Academy of Sciences (PNAS), a team of researchers at the UW-Madison School of Veterinary Medicine describes experiments that effectively promoted the ability of defective cells to take up and utilize an enzyme that is essential for the maintenance of a critical sheathing of nerve fibers.
The work centers on devising strategies to treat inherited diseases of the nervous system in which cells fail to maintain myelin, a protective sheathing that envelops nerve fibers and acts like the insulation on an electric wire. Myelin ensures the effective transmission of the signals routinely conducted by the nervous system. For those afflicted with Krabbe’s disease, the loss of myelin results in arrested motor and mental development, seizures, paralysis and, ultimately, death.
Ian Duncan | EurekAlert!
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