Target type: biologicalprocess
The chemical reactions and pathways resulting in the breakdown of glycosylceramides, any compound formed by the replacement of the glycosidic hydroxyl group of a cyclic form of a monosaccharide (or derivative) by a ceramide group. [GOC:ai]
Glycosylceramide catabolic process is a complex biological process that involves the breakdown of glycosylceramides, a type of sphingolipid found in cell membranes. This process is crucial for maintaining the integrity and function of cell membranes and plays a role in various cellular processes, including cell signaling, differentiation, and apoptosis.
**Key Steps in Glycosylceramide Catabolism:**
1. **Hydrolysis of the glycosidic bond:** The initial step involves the hydrolysis of the glycosidic bond that links the sugar moiety to the ceramide backbone. This reaction is catalyzed by a family of enzymes known as glycosidases, specifically glucosylceramidase (GBA) and galactosylceramidase (GALC).
2. **Ceramide degradation:** Once the glycosidic bond is cleaved, the ceramide moiety is further degraded through a series of enzymatic reactions. These reactions involve the removal of the fatty acid chain and the sphingosine base, ultimately leading to the production of simpler molecules such as sphingosine-1-phosphate (S1P).
**Enzymes Involved in Glycosylceramide Catabolism:**
* **Glucosylceramidase (GBA):** Catalyzes the hydrolysis of glucosylceramide, producing glucose and ceramide.
* **Galactosylceramidase (GALC):** Catalyzes the hydrolysis of galactosylceramide, producing galactose and ceramide.
* **Ceramidase:** Removes the fatty acid chain from ceramide, producing sphingosine.
* **Sphingosine kinase:** Phosphorylates sphingosine to produce S1P.
**Biological Significance:**
* **Membrane homeostasis:** Glycosylceramide catabolism plays a vital role in maintaining the fluidity and integrity of cell membranes.
* **Cell signaling:** S1P, a product of ceramide degradation, is a potent signaling molecule involved in various cellular processes, including cell growth, survival, and migration.
* **Apoptosis:** The breakdown of glycosylceramides can contribute to the initiation of apoptosis, a programmed cell death pathway.
* **Disease association:** Deficiencies in specific enzymes involved in glycosylceramide catabolism can lead to lysosomal storage disorders, such as Gaucher disease (GBA deficiency) and Krabbe disease (GALC deficiency).
**Regulation of Glycosylceramide Catabolism:**
The activity of enzymes involved in glycosylceramide catabolism is tightly regulated by factors such as substrate availability, pH, and the presence of inhibitors or activators. This regulation ensures that the breakdown of glycosylceramides occurs at appropriate levels to maintain cellular homeostasis.'
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Protein | Definition | Taxonomy |
---|---|---|
Cytosolic beta-glucosidase | A cytosolic beta-glucosidase that is encoded in the genome of human. [PRO:DNx, UniProtKB:Q9H227] | Homo sapiens (human) |
Lactase-phlorizin hydrolase | A lactase/phlorizin hydrolase that is encoded in the genome of human. [PRO:DNx, UniProtKB:P09848] | Homo sapiens (human) |
Alpha-galactosidase A | An alpha-galactosidase A that is encoded in the genome of human. [PRO:DNx, UniProtKB:P06280] | Homo sapiens (human) |
Compound | Definition | Classes | Roles |
---|---|---|---|
1-deoxynojirimycin | 1-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 |
miglustat | miglustat : 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 |
migalastat | migalastat: a potent inhibitor of glycolipid biosynthesis | piperidines | |
miglitol | piperidines | ||
2,5-dideoxy-2,5-imino-d-glucitol | 2,5-dideoxy-2,5-imino-D-glucitol: structure in first source | ||
validamine | validamine : 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-deoxynojirimycin | L-altro-1-deoxynojirimycin: structure in first source |