Target type: molecularfunction
Catalysis of the reaction: UDP-alpha-D-glucose + a dihydroceramide = UDP + H+ + a D-glucosyl-N-acylsphinganine. [EC:2.4.1.80, GOC:pz]
Dihydroceramide glucosyltransferase activity is a crucial step in the biosynthesis of glucosylceramide, a key sphingolipid component of cell membranes and a precursor to other important sphingolipids. This enzymatic activity catalyzes the transfer of a glucose molecule from UDP-glucose to a dihydroceramide molecule, resulting in the formation of glucosylceramide. The reaction involves the following steps:
1. **Substrate Binding:** Dihydroceramide glucosyltransferase binds both UDP-glucose and dihydroceramide at its active site.
2. **Glucose Transfer:** The enzyme facilitates the transfer of glucose from UDP-glucose to the C1-hydroxyl group of dihydroceramide.
3. **Product Release:** Glucosylceramide and UDP are released from the enzyme.
This enzymatic activity plays a critical role in various cellular functions, including:
* **Cell signaling:** Glucosylceramide, the product of this enzymatic activity, serves as a signaling molecule involved in various cellular processes, including cell growth, differentiation, and apoptosis.
* **Membrane structure and function:** Glucosylceramide is a major component of cellular membranes, contributing to their structural integrity and fluidity.
* **Lipid trafficking and storage:** Glucosylceramide is involved in the transport and storage of lipids within cells.
* **Immune responses:** Glucosylceramide plays a role in immune responses by modulating the activity of immune cells.
Dysregulation of dihydroceramide glucosyltransferase activity has been implicated in various diseases, including cancer, diabetes, and neurodegenerative disorders. Understanding the molecular mechanisms of this enzymatic activity is crucial for developing novel therapeutic strategies for these diseases.'
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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) |
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 |