Glucosylceramide (glucocerebroside) !

Glucosylceramide is a ceramide carrying one glucose. It is the lipid that builds up in macrophages in Gaucher disease.

Updated 2026-11-01

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Key facts

Ceramide + β-glucose at C1; the first step towards gangliosides and globosides.
Glucosylceramide synthase makes it in the Golgi (with UDP-glucose); eliglustat and miglustat inhibit it (substrate reduction).
Lysosomal glucocerebrosidase (GBA1), with saposin C, breaks it into glucose + ceramide.
Macrophages receive it mostly from the membranes of old red and white cells they engulf.

The 3D model (PDB 03F) is a β-glucosylceramide with oleic acid (C18:1) on the nitrogen.
The β-linked glucose is what sets this cerebroside apart from myelin galactosylceramide.

In Gaucher disease it accumulates in macrophages of spleen, liver and marrow (Gaucher cells, with "crumpled tissue paper" cytoplasm).
Its deacylated form, glucosylsphingosine (lyso-Gb1), is the blood biomarker used to follow the disease.

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Which enzyme breaks down glucosylceramide, and in which organelle?

Glucocerebrosidase (acid β-glucosidase, GBA1), inside the lysosome.

How does substrate reduction therapy work in Gaucher disease?

It inhibits glucosylceramide synthase, so less lipid is made to accumulate.

Where does most of the glucosylceramide in Gaucher macrophages come from?

From the membranes of old blood cells that macrophages engulf.

Molecules are shown in their neutral form, as the databases deposit them; in the cell the acids and phosphates are ionised.

Educational information, not medical advice. For any symptom, see a health professional.

Sources: RCSB PDB - 03F Ligand Summary Page (RCSB Protein Data Bank, 2026); Glycosphingolipid metabolism (Reactome, 2026); GBA1 gene: MedlinePlus Genetics (MedlinePlus, US National Library of Medicine, 2026); Textbook of Biochemistry for Medical Students, 6th ed. (Jaypee Brothers Medical Publishers (Vasudevan DM, Sreekumari S, Vaidyanathan K), 2011).

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Representative model, for educational purposes only.