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

# GM2 ganglioside

GM2 ganglioside is a sialic acid-containing glycolipid of neuronal membranes. It builds up in neurons in Tay–Sachs disease.

Updated 2026-11-01

## Key facts

- Ceramide + glucose + galactose + N-acetylgalactosamine, with one sialic acid (N-acetylneuraminic acid) on the galactose.
- "G" = ganglioside, "M" = one (mono) sialic acid, "2" = its chromatography migration order.
- Hexosaminidase A (αβ) removes the terminal N-acetylgalactosamine, turning it into GM3; it needs the GM2 activator protein.
- It fails in Tay–Sachs (α subunit, HEXA), Sandhoff (β subunit, HEXB) and the AB variant (activator, GM2A).

**Also called**
GM2, Tay–Sachs ganglioside

**Formula**
C₆₈H₁₂₃N₃O₂₆

## Structure

- PubChem (CID 71308211) offers no 3D model because of its size and flexibility, and there is no PDB chemical component for the whole ganglioside; this page has no 3D.
- A four-sugar head with a negative charge (sialic acid) on two ceramide tails that sink into the bilayer.

## Clinical notes

- In Tay–Sachs, neurons swell with GM2 (membranous cytoplasmic bodies): regression, seizures, blindness and a cherry-red spot.
- In Sandhoff disease globoside also accumulates in organs, so hepatosplenomegaly may appear.

## Pathways

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

## Related conditions

- [Tay–Sachs disease](https://durgical.com/en/conditions/tay-sachs-disease/)

## Related

- [Hexosaminidase A (β-hexosaminidase A)](https://durgical.com/en/biochemistry/enzymes/hexosaminidase-a/)
- [Sphingolipids and sphingolipidoses](https://durgical.com/en/biochemistry/topics/sphingolipids-and-sphingolipidoses/)
- [Ceramide](https://durgical.com/en/biochemistry/molecules/ceramide/)
- [Glucosylceramide (glucocerebroside)](https://durgical.com/en/biochemistry/molecules/glucosylceramide/)

## Test yourself

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

## Which sugar does hexosaminidase A remove from GM2 ganglioside?

The terminal N-acetylgalactosamine; the product is GM3 ganglioside.

## What do the letters and number in "GM2" mean?

G = ganglioside, M = a single sialic acid, 2 = its chromatographic migration order.

## Which three genetic defects store GM2?

HEXA (Tay–Sachs), HEXB (Sandhoff) and GM2A (AB variant, missing activator).

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

Sources: [Ganglioside GM2 (CID 71308211)](https://pubchem.ncbi.nlm.nih.gov/compound/71308211) (PubChem, NCBI, 2026) · Public domain; [Glycosphingolipid metabolism](https://reactome.org/PathwayBrowser/#/R-HSA-1660662) (Reactome, 2026); [HEXA gene: MedlinePlus Genetics](https://medlineplus.gov/genetics/gene/hexa/) (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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Source: https://durgical.com/en/biochemistry/molecules/gm2-ganglioside/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/biochemistry/molecules/gm2-ganglioside/