[Durgical](https://durgical.com/en/) › [DurgiPedia](https://durgical.com/en/durgipedia/) › [Biochemistry](https://durgical.com/en/biochemistry/) › Globotriaosylceramide (Gb3)

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

# Globotriaosylceramide (Gb3)

Gb3 is a ceramide carrying three sugars. α-Galactosidase A breaks it down; in Fabry disease it builds up in vessels, kidney, heart and nerves.

Updated 2026-11-01

## Key facts

- Structure: ceramide–glucose–galactose–α-galactose (Galα1→4Galβ1→4Glc-ceramide); α-galactosidase A removes the terminal α-galactose.
- Fabry disease (GLA gene, X-linked): Gb3 and its deacylated form lyso-Gb3 accumulate in endothelium, podocytes, cardiomyocytes and nerve cells.
- Plasma lyso-Gb3 is a biomarker that supports the diagnosis of Fabry disease and helps follow treatment.
- Gb3 is the cell receptor for Shiga toxin (and the Shiga-like toxins of E. coli O157:H7), which explains the kidney damage of haemolytic uraemic syndrome.

**Also called**
Gb3, Ceramide trihexoside, CD77

**Formula**
C₆₀H₁₁₃NO₁₈ (C24:0 form; it varies with the fatty acid)

## Structure

- No 3D model: there is no PubChem 3D conformer or PDB chemical component for whole Gb3, a large and flexible lipid.
- The trisaccharide head faces out of the membrane and the two hydrocarbon tails anchor it; the formula changes with the fatty acid (C16 to C24).
- It is made from lactosylceramide by adding α-galactose (Gb3 synthase, A4GALT) and is extended to globoside (Gb4) by adding N-acetylgalactosamine.

## Clinical notes

- Fabry disease: burning pain in hands and feet, angiokeratomas, reduced sweating, corneal whorls, proteinuria progressing to kidney failure, left ventricular hypertrophy and stroke.
- Women with one altered GLA copy can have symptoms, often milder, because of X inactivation.
- Enzyme replacement, and an oral chaperone for amenable GLA variants, lower Gb3 storage.

## Related conditions

- [Fabry disease](https://durgical.com/en/conditions/fabry-disease/)

## Related

- [α-Galactosidase A](https://durgical.com/en/biochemistry/enzymes/galactosidase-a/)
- [Ceramide](https://durgical.com/en/biochemistry/molecules/ceramide/)
- [Glucosylceramide (glucocerebroside)](https://durgical.com/en/biochemistry/molecules/glucosylceramide/)
- [Sphingolipids and sphingolipidoses](https://durgical.com/en/biochemistry/topics/sphingolipids-and-sphingolipidoses/)
- [Lysosomal storage diseases](https://durgical.com/en/biochemistry/topics/lysosomal-storage-diseases/)
- [Hexosaminidase B (β-hexosaminidase B)](https://durgical.com/en/biochemistry/enzymes/hexosaminidase-b/)
- [Galactose](https://durgical.com/en/biochemistry/molecules/galactose/)

## Test yourself

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

## Which enzyme breaks down globotriaosylceramide?

α-Galactosidase A (GLA gene), which removes the terminal α-galactose; its deficiency causes Fabry disease.

## Which Gb3-derived molecule is measured in plasma as a Fabry biomarker?

Lyso-Gb3 (globotriaosylsphingosine), Gb3 without its fatty acid.

## Why can Shiga toxin damage the kidney?

It binds Gb3 on the surface of renal and endothelial cells, enters them and stops protein synthesis, leading to haemolytic uraemic syndrome.

## Why does this page have no 3D model?

Whole Gb3 has no PubChem 3D conformer and no PDB chemical component; it is too large and flexible for those libraries.

[Add to my cards (4)](https://durgical.app/estudiar/?add=/en/biochemistry/molecules/globotriaosylceramide/)

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

Sources: [GLA gene: MedlinePlus Genetics](https://medlineplus.gov/genetics/gene/gla/) (MedlinePlus, US National Library of Medicine, 2026); [Fabry disease: MedlinePlus Genetics](https://medlineplus.gov/genetics/condition/fabry-disease/) (MedlinePlus, US National Library of Medicine, 2026); [Glycosphingolipid catabolism](https://reactome.org/PathwayBrowser/#/R-HSA-9840310) (Reactome, 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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