Page last updated: 2024-10-24

glycosylceramidase activity

Definition

Target type: molecularfunction

Catalysis of the reaction: glycosyl-N-acylsphingosine + H2O = a sugar + N-acylsphingosine. [PMID:10692580, PMID:9762914]

Glycosylceramidase activity is a hydrolytic enzyme activity that catalyzes the breakdown of glycosylceramides, which are lipids found in cell membranes. These enzymes specifically target the glycosidic bond between a sugar moiety and ceramide, releasing a free sugar and ceramide. This hydrolysis reaction plays a crucial role in various biological processes, including:

1. **Sphingolipid metabolism:** Glycosylceramidase activity is essential for the breakdown and recycling of sphingolipids, a class of lipids that play important roles in cell signaling, membrane structure, and cell growth.

2. **Lysosomal function:** Many glycosylceramidases reside within lysosomes, the cellular organelles responsible for degrading waste products. These enzymes break down glycosylceramides that have accumulated in lysosomes, preventing their buildup and potential cellular dysfunction.

3. **Cellular signaling:** Some glycosylceramidases participate in cellular signaling pathways by modulating the levels of specific sphingolipid species, which act as second messengers.

4. **Immune function:** Glycosylceramidase activity is involved in the immune response by regulating the production and activity of certain immune cells.

5. **Nervous system function:** Glycosylceramidase activity is crucial for maintaining the integrity of the myelin sheath, a protective covering around nerve fibers. Defects in glycosylceramidases can lead to neurological disorders.

In summary, glycosylceramidase activity is a vital enzymatic process that contributes to multiple biological functions. It is essential for the breakdown of glycosylceramides, influencing sphingolipid metabolism, lysosomal function, cellular signaling, immune responses, and nervous system function.'
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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)
Lactase-phlorizin hydrolaseA lactase/phlorizin hydrolase that is encoded in the genome of human. [PRO:DNx, UniProtKB:P09848]Homo sapiens (human)

Compounds (8)

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
miglustatmiglustat : A hydroxypiperidine that is deoxynojirimycin in which the amino hydrogen is replaced by a butyl group.

miglustat: a glucosylceramide synthase inhibitor
piperidines;
tertiary amino compound
anti-HIV agent;
EC 2.4.1.80 (ceramide glucosyltransferase) inhibitor
migalastatmigalastat: a potent inhibitor of glycolipid biosynthesispiperidines
miglitolpiperidines
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-(5-adamantane-1-yl-methoxy-pentyl)deoxynojirimycin
l-altro-1-deoxynojirimycinL-altro-1-deoxynojirimycin: structure in first source