Page last updated: 2024-10-24

galactosylceramide catabolic process

Definition

Target type: biologicalprocess

The chemical reactions and pathways resulting in the breakdown of galactosylceramides, any compound formed by the replacement of the glycosidic hydroxyl group of a cyclic form of galactose by a ceramide group. [GOC:ai]

Galactosylceramide catabolic process is the breakdown of galactosylceramide, a sphingolipid found in the myelin sheath that insulates nerve cells. This process involves the sequential action of several enzymes. First, galactosylceramide is hydrolyzed by the enzyme galactosylceramidase (also known as beta-galactosidase) into galactose and ceramide. Ceramide is then further broken down by ceramidase into sphingosine and fatty acid. Galactose is then metabolized through the galactose metabolism pathway. This pathway starts with the conversion of galactose to galactose-1-phosphate by the enzyme galactokinase. Galactose-1-phosphate is then converted to UDP-galactose by the enzyme UDP-galactose-4-epimerase. UDP-galactose can then be used in various metabolic pathways, including glycolysis, glycogen synthesis, and lactose synthesis. The catabolism of galactosylceramide is essential for maintaining the integrity of the myelin sheath and ensuring proper nerve function. Mutations in the genes encoding the enzymes involved in galactosylceramide catabolism can lead to various neurological disorders, such as Krabbe disease.'
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Proteins (2)

ProteinDefinitionTaxonomy
Cytosolic beta-glucosidaseA cytosolic beta-glucosidase that is encoded in the genome of human. [PRO:DNx, UniProtKB:Q9H227]Homo sapiens (human)
GalactocerebrosidaseA galactocerebrosidase that is encoded in the genome of human. [PRO:DNx, UniProtKB:P54803]Homo sapiens (human)

Compounds (4)

CompoundDefinitionClassesRoles
1-deoxynojirimycin1-deoxy-nojirimycin: structure in first source

duvoglustat : An optically active form of 2-(hydroxymethyl)piperidine-3,4,5-triol having 2R,3R,4R,5S-configuration.
2-(hydroxymethyl)piperidine-3,4,5-triol;
piperidine alkaloid
anti-HIV agent;
anti-obesity agent;
bacterial metabolite;
EC 3.2.1.20 (alpha-glucosidase) inhibitor;
hepatoprotective agent;
hypoglycemic agent;
plant metabolite
2,5-dideoxy-2,5-imino-d-glucitol2,5-dideoxy-2,5-imino-D-glucitol: structure in first source
validaminevalidamine : An amino cyclitol consisting of 1D-chiro-inositol lacking the 6-hydroxy group and having those at positions 1 and 5 replaced by amino and hydroxymethyl groups respectively.

validamine: RN given from CA Index Guide; RN not in Chemline 11/84; structure given in first source
amino cyclitol
n-nonyl-1-deoxynojirimycinN-nonyldeoxynojirimycin : A hydroxypiperidine that is deoxynojirimycin (duvoglustat) in which the amino hydrogen is replaced by a nonyl group.hydroxypiperidine;
tertiary amino compound
antiviral agent;
EC 3.2.1.20 (alpha-glucosidase) inhibitor;
EC 3.2.1.45 (glucosylceramidase) inhibitor