Krabbe Disease: Lipid Accumulation
Krabbe disorder, also called globoid cells leukodystrophy or globoid cell syndrome, is a rare condition that can affect the nervous system. It is caused by a deficiency in the enzyme galactocerebrosidase, which is responsible for breaking down certain lipids, including ceramide, sphingosine, galactosphingosine, and galactosylceramide (Kumar & Dutta, 2020). This deficiency causes lipid accumulation in the body which can cause nerve cell damage.
These lipids are a hallmark of Krabbe Disease (Brennen and colleagues, 2018). Galactosylceramide and ceramide were the most common lipids found in Krabbe patients (Tian, 2021). Galactosphingosine, sphingosine, and ceramide have been also identified as lipids found in Krabbe Disease (Moreau, et al. 2016).
The lipids Krabbe disease accumulates include ceramide and sphingosine as well as galactosphingosine and galactosylceramide. These lipids accumulate in Krabbe disease and are a sign of deficiency. Cont….
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