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Biochemistry · Molecule

Sulfatide !

Sulfatide is a galactosylceramide with a sulfate group. It is part of myelin and builds up in metachromatic leukodystrophy.

Updated 2026-11-01

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Key facts

  • Galactosylceramide + sulfate at C3 of the galactose (donated by PAPS).
  • Abundant in central and peripheral myelin, and in the kidney.
  • Arylsulfatase A (ARSA gene), with saposin B, removes the sulfate in the lysosome.
  • It stains a colour different from the dye (metachromasia) with toluidine blue or cresyl violet, hence the disease's name.
Also called
Sulfogalactosylceramide, 3-O-sulfogalactosylceramide
Formula
C₄₈H₉₃NO₁₂S

Structure

  • The 3D model (PDB SFT) is a sulfogalactosylceramide with a 24-carbon chain.
  • The sulfate gives the head a strong negative charge.

Clinical notes

  • In metachromatic leukodystrophy, sulfatides accumulate in oligodendrocytes, Schwann cells, kidney and gallbladder, and appear in urine.
  • Saposin B deficiency gives a similar picture with normal arylsulfatase A activity.

Test yourself

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

Which enzyme removes the sulfate from sulfatide?

Arylsulfatase A (ARSA gene), helped by saposin B.

Why is metachromatic leukodystrophy so named?

Stored sulfatides stain a colour different from the dye used (metachromasia).

Which molecule donates the sulfate to make sulfatides?

PAPS (3'-phosphoadenosine 5'-phosphosulfate).

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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 - SFT Ligand Summary Page (RCSB Protein Data Bank, 2026); Glycosphingolipid metabolism (Reactome, 2026); ARSA gene: MedlinePlus Genetics (MedlinePlus, US National Library of Medicine, 2026); Metachromatic leukodystrophy: 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.