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[Biochemistry](https://durgical.com/en/biochemistry/) · Molecule

# 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.

**Also called**
Glucocerebroside, GlcCer

**Formula**
C₄₂H₇₉NO₈

## Structure

- 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.

## Clinical notes

- 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.

## Pathways

- [Lysosomal ganglioside breakdown](https://durgical.com/en/biochemistry/pathways/lysosomal-ganglioside-breakdown/)

## Related conditions

- [Gaucher disease](https://durgical.com/en/conditions/gaucher-disease/)

## Related

- [Glucocerebrosidase (acid β-glucosidase)](https://durgical.com/en/biochemistry/enzymes/glucocerebrosidase/)
- [Ceramide](https://durgical.com/en/biochemistry/molecules/ceramide/)
- [Sphingolipids and sphingolipidoses](https://durgical.com/en/biochemistry/topics/sphingolipids-and-sphingolipidoses/)
- [Galactosylceramide (galactocerebroside)](https://durgical.com/en/biochemistry/molecules/galactosylceramide/)
- [Glucose](https://durgical.com/en/biochemistry/molecules/glucose/)

## Test yourself

Think of the answer, then tap the question to see it.

## 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.

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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](https://www.rcsb.org/ligand/03F) (RCSB Protein Data Bank, 2026); [Glycosphingolipid metabolism](https://reactome.org/PathwayBrowser/#/R-HSA-1660662) (Reactome, 2026); [GBA1 gene: MedlinePlus Genetics](https://medlineplus.gov/genetics/gene/gba1/) (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.

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