Target type: molecularfunction
Catalysis of the reaction: UDP-glucose + N-acylsphingosine = UDP + D-glucosyl-N-acylsphingosine. [EC:2.4.1.80]
Ceramide glucosyltransferase activity is a crucial enzymatic process involved in the synthesis of glucosylceramide (GlcCer), a vital sphingolipid found in eukaryotic cell membranes. This enzyme catalyzes the transfer of glucose from UDP-glucose to the primary hydroxyl group of ceramide, forming GlcCer. This reaction plays a critical role in several cellular processes, including:
* **Sphingolipid metabolism:** GlcCer is a precursor for the biosynthesis of more complex glycosphingolipids, which are essential components of cell membranes and play a role in cell signaling, recognition, and adhesion.
* **Cell signaling:** GlcCer itself can act as a signaling molecule, regulating various cellular processes such as cell growth, differentiation, and apoptosis.
* **Membrane organization:** GlcCer contributes to the formation of lipid rafts, specialized microdomains within cell membranes that are enriched in sphingolipids and cholesterol. These rafts are important for the compartmentalization and regulation of various cellular processes.
* **Disease pathogenesis:** Abnormal levels of GlcCer have been linked to various diseases, including Gaucher disease, Fabry disease, and cancer.
In summary, ceramide glucosyltransferase activity is a key enzymatic step in the biosynthesis of GlcCer, a crucial sphingolipid with diverse functions in cell biology and disease.'
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Protein | Definition | Taxonomy |
---|---|---|
Ceramide glucosyltransferase | A ceramide glucosyltransferase that is encoded in the genome of human. [PRO:DNx, UniProtKB:Q16739] | Homo sapiens (human) |
Ceramide glucosyltransferase | A ceramide glucosyltransferase that is encoded in the genome of human. [PRO:DNx, UniProtKB:Q16739] | 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 | ||
n-(5-adamantane-1-yl-methoxy-pentyl)deoxynojirimycin | |||
l-altro-1-deoxynojirimycin | L-altro-1-deoxynojirimycin: structure in first source |