Agalsidase α
The pharmaceutical company Shire manufactures agalsidase alfa (INN) under the trade name Replagal as a treatment for Fabry disease, and was granted marketing approval in the EU in 2001. FDA approval was applied for the United States. However, in 2012, Shire withdrew their application for approval in the United States citing that the agency will require additional clinical trials before approval. Agalsidase β
The pharmaceutical company Genzyme produces synthetic agalsidase β (INN) under the trade name Fabrazyme for treatment of Fabry disease. In 2009, contamination at Genzymes 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 companys patent on the drug under the "march-in" provisions of the Bayh–Dole Act. Pharmacological chaperone therapy
Fabry patients who display neurological symptoms cannot receive RERT because recombinant enzymes cannot normally pass the blood-brain barrier. Thus, a more suitable alternative treatment is used: pharmacological chaperone therapy. It has been shown that more potent competitive inhibitors of an enzyme can act as a more powerful chemical chaperone for the corresponding mutant enzyme that fails to maintain proper folding and conformation, despite its intact active site. These chemical chaperones bind to the active site of the mutant enzyme, which can help promote proper folding and stabilize the mutant enzyme. Thus, this results in functional mutant enzymes that will not be degraded via the ubiquitin-proteasome pathway.
Supplements
α-GAL derived from the mold Aspergillus niger is an active ingredient in products marketed to reduce stomach gas production after eating foods known to cause gas. It is optimally active at 55°C, after which its half-life is 120 minutes.There are scores of supplements containing the enzyme over the counter in the United States and many more world wide. Products with α-galactosidase include:

Beano
CVS BeanAid
Enzymedicas BeanAssist
Gasfix
Bloateez (in India as Cogentrix)

See also
β-galactosidase
Migalastat, a drug targeting α-galactosidase
Classification of α-galactosidases (according to CAZy)

References
Further reading
External links
alpha-Galactosidase at the US National Library of Medicine Medical Subject Headings (MeSH)
Human GLA genome location and GLA gene details page  in the UCSC Genome Browser.This article incorporates text from the United States National Library of Medicine, which is in the public domain.