Research on enzyme replacement in Krabbe disease

University of Wisconsin-Madison researchers have authored a study of cellular activity in Krabbe disease, a neurological disorder. Their research, published in the December 12, 2005, issue of Proceedings of the National Academy of Sciences, explored how cells obtained from a mouse model of Krabbe disease could be reinvigorated by replacing a missing enzyme, galactocerebrosidase. Without the enzyme, the myelin sheath around nerve fibers can't be maintained. When supplied with galactocerebrosidase, the defective cells from the Krabbe disease mouse took up the enzyme and sparked widespread and persistent myelination of the mouse brain and spinal cord. This finding may provide the basis for new treatment strategies for Krabbe disease. The research was funded by Hunter's Hope.