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UDP-galactose:ceramide galactosyltransferase (UGT8) is a class I integral membrane enzyme located in the endoplasmic reticulum. It catalyzes the transfer of galactose from UDP-galactose to ceramide, producing galactosylceramide—the major sphingolipid component of myelin in the central and peripheral nervous systems. This enzymatic activity is essential for proper myelination of neurons. UGT8 is also found in some epithelial tissues and has roles in lipid and protein sorting. Deficits or misregulation of this enzyme are implicated in neurological disorders (such as Krabbe disease) and certain cancers (for example, as a molecular marker for aggressive breast cancer)
Inhibition of UGT8 blocks galactocerebroside synthesis
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