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A galactosylceramide, or galactocerebroside is a type of cerebroside consisting of a ceramide with a galactose residue at the 1-hydroxyl moiety. The galactose is cleaved by galactosylceramidase . Galactosylceramide is a marker for oligodendrocytes in the brain, whether or not they form myelin .
The fundamental structure of a cerebroside is ceramide.Monoglycosyl and oligoglycosylceramides having a mono or polysaccharide bonded glycosidically to the terminal OH group of ceramide are defined as cerebrosides.
This reaction occurs in the luminal leaflet of the endoplasmic reticulum, and its final product is GalCer, or galactocerebroside, which is then transported to the Golgi apparatus. [1] [2] Here, GalCer reacts with 3’-phosphoadenosine-5’-phosphosulfate to make sulfatide. This reaction is catalyzed by cerebroside sulfotransferase (CST). [1]
Galactosylceramidase (or galactocerebrosidase), EC 3.2.1.46, is an enzyme that removes galactose from ceramide derivatives (galactosylceramides) by catalysing the hydrolysis of galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride.
Krabbe disease (KD) (also known as globoid cell leukodystrophy [3] or galactosylceramide lipidosis) is a rare and often fatal lysosomal storage disease that results in progressive damage to the nervous system.
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GM1 (monosialotetrahexosylganglioside) the "prototype" ganglioside, is a member of the ganglio series of gangliosides which contain one sialic acid residue. GM1 has ...
The galactosphingolipid galactocerebroside (GalC) and its sulfated derivative sulfatide is also in abundance present (together with a small group of proteins) in myelin, the membrane around the axons in the nervous system of vertebrates. [6]