Galactosylceramide (galactocerebroside) !

Galactosylceramide is a ceramide with one galactose and a main myelin lipid. Failure to break it down causes Krabbe disease.

Updated 2026-11-01

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Key facts

Ceramide + β-galactose; made by oligodendrocytes and Schwann cells.
With a sulfate on the galactose it becomes sulfatide.
Galactocerebrosidase (GALC gene) breaks it into galactose + ceramide in the lysosome.
The same enzyme breaks down psychosine (galactosylsphingosine), a lipid toxic to myelin-forming cells.

The 3D model (PDB GCY) is a galactosylceramide with a 24-carbon fatty acid (lignoceric acid), typical of myelin.
It differs from glucosylceramide only in the sugar: galactose (a C4 epimer) instead of glucose.

In Krabbe disease, stored psychosine kills oligodendrocytes: fast demyelination and multinucleated globoid cells.
Antibodies against galactocerebroside have been studied in demyelinating neuropathies.

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

Which enzyme is missing in Krabbe disease?

Galactocerebrosidase (GALC), which breaks down galactosylceramide and psychosine.

Which toxic lipid explains oligodendrocyte death in Krabbe disease?

Psychosine (galactosylsphingosine), which is not broken down without GALC.

How do galactosylceramide and glucosylceramide differ?

Only in the sugar on the ceramide: galactose versus glucose, C4 epimers.

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 - GCY Ligand Summary Page (RCSB Protein Data Bank, 2026); Glycosphingolipid metabolism (Reactome, 2026); Krabbe disease: MedlinePlus Genetics (MedlinePlus, US National Library of Medicine, 2026); GALC 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.