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

# Glucocerebrosidase (acid β-glucosidase)

Glucocerebrosidase is the lysosomal enzyme that removes glucose from glucosylceramide. Its absence causes Gaucher disease.

Updated 2026-11-01

Loading the 3D model…

## Key facts

- Glucosylceramide + H₂O → glucose + ceramide, at acid pH inside the lysosome.
- It needs saposin C (from the PSAP gene) to reach the lipid on the inner lysosomal membrane.
- It does not use mannose 6-phosphate: it reaches the lysosome with the membrane protein LIMP-2.
- Single-allele GBA1 variants are the most common genetic risk factor for Parkinson disease.

**Also called**
Acid β-glucosylceramidase, GCase

**EC number**
[3.2.1.45](https://www.enzyme-database.org/query.php?ec=3.2.1.45)

**Genes**
GBA1

**Structure (PDB)**
[2V3D](https://www.rcsb.org/structure/2V3D)

## Regulation

- Active at acid pH (≈ 5), inactive at the neutral pH of the cytosol.
- The transcription factor TFEB increases its synthesis along with other lysosomal enzymes.

## Inhibitors and toxins

- Miglustat (N-butyl-deoxynojirimycin), the ligand in the 3D model; clinically it acts mainly by slowing glucosylceramide synthase
- Isofagomine (pharmacological chaperone under study)
- Conduritol B epoxide (irreversible laboratory inhibitor)

## Clinical notes

- Its deficiency causes Gaucher disease (types 1, 2 and 3); the N370S (N409S) variant gives type 1 and protects against neurological damage.
- Recombinant replacement enzyme is modified to expose mannoses and enter macrophages through the mannose receptor.
- Carriers of GBA1 variants have a higher risk of Parkinson disease and dementia with Lewy bodies.

## Pathways

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

## Related conditions

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

## Related

- [Glucosylceramide (glucocerebroside)](https://durgical.com/en/biochemistry/molecules/glucosylceramide/)
- [Ceramide](https://durgical.com/en/biochemistry/molecules/ceramide/)
- [Sphingolipids and sphingolipidoses](https://durgical.com/en/biochemistry/topics/sphingolipids-and-sphingolipidoses/)
- [Lysosomal storage diseases](https://durgical.com/en/biochemistry/topics/lysosomal-storage-diseases/)
- [Mannose 6-phosphate tagging (sending enzymes to the lysosome)](https://durgical.com/en/biochemistry/topics/mannose-6-phosphate-tagging/)

## Test yourself

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

## Which reaction does glucocerebrosidase catalyse?

It hydrolyses glucosylceramide into glucose and ceramide inside the lysosome.

## Which activator protein does glucocerebrosidase need?

Saposin C, which lifts the lipid from the membrane and presents it to the enzyme.

## Why is Gaucher replacement enzyme designed with exposed mannoses?

So macrophages, the cells that store the lipid, take it up through their mannose receptor.

## Which common neurodegenerative disease is linked to GBA1 variants?

Parkinson disease: GBA1 variants are its most frequent genetic risk factor.

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

Sources: [GBA1: Lysosomal acid glucosylceramidase (human)](https://www.uniprot.org/uniprotkb/P04062/entry) (UniProt, 2026); [GBA1 gene: MedlinePlus Genetics](https://medlineplus.gov/genetics/gene/gba1/) (MedlinePlus, US National Library of Medicine, 2026); [2V3D: acid-beta-glucosidase with N-butyl-deoxynojirimycin](https://www.rcsb.org/structure/2V3D) (RCSB Protein Data Bank, 2026); [Glycosphingolipid metabolism](https://reactome.org/PathwayBrowser/#/R-HSA-1660662) (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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