Available structures
PDBOrtholog search: PDBe RCSB
Aliases GLA, GALA, galactosidase alpha
External IDs MGI: 1347344 HomoloGene: 90852 GeneCards: GLA
RNA expression pattern
More reference expression data
Species Human Mouse









RefSeq (mRNA)



RefSeq (protein)



Location (UCSC) Chr X: 101.4 – 101.41 Mb Chr X: 134.59 – 134.6 Mb
PubMed search [1] [2]
View/Edit HumanView/Edit Mouse
EC number
CAS number 9025-35-8
IntEnz IntEnz view
ExPASy NiceZyme view
MetaCyc metabolic pathway
PRIAM profile
PDB structures RCSB PDB PDBe PDBsum
Gene Ontology AmiGO / EGO

Alpha-galactosidase is a glycoside hydrolase enzyme that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. It is encoded by the GLA gene.[3] Two recombinant forms of alpha-galactosidase are called agalsidase alfa (INN) and agalsidase beta (INN).


This enzyme is a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. It predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose.


A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry's disease, a rare lysosomal storage disorder and sphingolipidosis that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties.[4]

Two enzyme replacement therapies are available to functionally compensate for alpha-galactosidase deficiency. Agalsidase alpha and beta are both recombinant forms of the human α-galactosidase A enzyme and both have the same amino acid sequence as the native enzyme. Agalsidase alpha and beta differ in the structures of their oligosaccharide side chains.[5]

Agalsidase alfa

The pharmaceutical company Shire manufactures agalsidase alfa (INN) under the trade name Replagal as a treatment for Fabry's disease,[6] and was granted marketing approval in the EU in 2001.[7] FDA approval was applied for the United States.[8] However, in 2012, Shire withdrew their application for approval in the United States citing that the agency will require additional clinical trials before approval.[9]

Agalsidase beta

The pharmaceutical company Genzyme produces synthetic agalsidase beta (INN) under the trade name Fabrazyme for treatment of Fabry's disease. In 2009, contamination at Genzyme's Allston, Massachusetts plant caused a worldwide shortage of Fabrazyme, and supplies were rationed to patients at one-third the recommended dose. Some patients have petitioned to break the company's patent on the drug under the "march-in" provisions of the Bayh–Dole Act.[8]

Over-the-counter brand names

Alpha-galactosidase is an active ingredient in Beano, Suntaqzyme, Bean-zyme, and Gas-zyme 3x, Bloateez (India) marketed as products to reduce stomach gas production after eating foods known to cause gas. There are dozens of generic brands containing the enzyme in the United States. It is optimally active at 55 degrees C, after which its half-life is 120 minutes.[10]

See also


  1. "Human PubMed Reference:".
  2. "Mouse PubMed Reference:".
  3. Calhoun DH, Bishop DF, Bernstein HS, Quinn M, Hantzopoulos P, Desnick RJ (1985). "Fabry disease: isolation of a cDNA clone encoding human alpha-galactosidase A". Proceedings of the National Academy of Sciences of the United States of America. 82 (21): 7364–8. Bibcode:1985PNAS...82.7364C. doi:10.1073/pnas.82.21.7364. PMC 391345Freely accessible. PMID 2997789.
  4. "Entrez Gene: GLA galactosidase, alpha".
  5. Fervenza FC, Torra R, Warnock DG (December 2008). "Safety and efficacy of enzyme replacement therapy in the nephropathy of Fabry disease". Biologics. 2 (4): 823–43. doi:10.2147/btt.s3770. PMC 2727881Freely accessible. PMID 19707461.
  6. Keating GM (October 2012). "Agalsidase alfa: a review of its use in the management of Fabry disease". BioDrugs. 26 (5): 335–54. doi:10.2165/11209690-000000000-00000. PMID 22946754.
  7. "Shire Submits Biologics License Application (BLA) for REPLAGAL with the U.S. Food and Drug Administration (FDA)". FierceBiotech.
  8. 1 2 "With A Life-Saving Medicine In Short Supply, Patients Want Patent Broken". 2010-08-04. Archived from the original on 14 September 2010. Retrieved 2010-09-02.
  9. Grogan K (2012-03-15). "Shire withdraws Replagal in USA as FDA wants more trials". PharmaTimes.
  10. Patil AG, K PK, Mulimani VH, Veeranagouda Y, Lee K (2010). "alpha-Galactosidase from Bacillus megaterium VHM1 and its application in removal of flatulence-causing factors from soymilk". Journal of Microbiology and Biotechnology. 20 (11): 1546–54. doi:10.4014/jmb.0912.12012. PMID 21124061.

Further reading

External links

This article incorporates text from the United States National Library of Medicine, which is in the public domain.

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