Ceramide glucosyltransferase (or glucosylceramide synthase) is a
glucosyltransferase Glucosyltransferases are a type of glycosyltransferase that enable the transfer of glucose.
Examples include:
* glycogen synthase
* glycogen phosphorylase
Glycogen phosphorylase is one of the phosphorylase enzymes (). Glycogen phosphorylase c ...
enzyme involved in the production of
glucocerebroside
Glucocerebroside (also called glucosylceramide) is any of the cerebrosides in which the monosaccharide head group is glucose.
Clinical significance
In Gaucher disease, the enzyme glucocerebrosidase is nonfunctional and cannot break down glucoce ...
.
It is classified under .
It is inhibited by
miglustat
Miglustat, sold under the brand name Zavesca, is a medication used to treat type I Gaucher disease (GD1). It is also known as N-butyldeoxynojirimycin, and is a derivative of the anti-diabetic 1-deoxynojirimycin. It was developed by Oxford GlycoSc ...
and
eliglustat, both drugs developed for the treatment of
Gaucher disease
Gaucher's disease or Gaucher disease () (GD) is a genetic disorder in which glucocerebroside (a sphingolipid, also known as glucosylceramide) accumulates in cells and certain organs. The disorder is characterized by bruising, fatigue, anemia, low ...
.
See also
*
Glucocerebroside
Glucocerebroside (also called glucosylceramide) is any of the cerebrosides in which the monosaccharide head group is glucose.
Clinical significance
In Gaucher disease, the enzyme glucocerebrosidase is nonfunctional and cannot break down glucoce ...
References
External links
*
Transferases
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