Publications by authors named "Joseph Elsbernd"
Proc Natl Acad Sci U S A
October 2019
Article Synopsis
- Infantile globoid cell leukodystrophy (GLD), or Krabbe disease, is a serious condition caused by a lack of the enzyme galactosylceramidase (GALC), leading to toxic buildup of a glycolipid called psychosine.
- New research indicates that psychosine is produced from the breakdown of galactosylceramide by the enzyme acid ceramidase (ACDase), not through the expected anabolic process, thus separating GALC deficiency from psychosine buildup.
- Inhibition of ACDase has been shown to reduce psychosine levels and improve outcomes in animal models, suggesting it could be a promising target for new therapies for Krabbe disease, offering a potentially safer alternative compared to
