However, a small number of females carrying only one copy of the mutated GLA gene never shows any symptoms of Fabry disease. Cause
Mutations to the GLA gene encoding α-GAL may result in complete loss of function of the enzyme. α-GAL is a lysosomal protein responsible for breaking down globotriaosylceramide, a fatty substance stored various types of cardiac and renal cells. When globotriaosylceramide is not properly catabolized, it is accumulated in cells lining blood vessels in the skin, cells in the kidney, heart and nervous system. As a result, signs and symptoms of Fabry disease begin to manifest. Treatment
There are three treatment options for Fabry disease: recombinant enzyme replacement therapy, pharmacological chaperone therapy, and organ specific treatment. Recombinant enzyme replacement therapy (RERT)
RERT was approved as a treatment for Fabry disease in the United States in 2003.Two recombinant enzyme replacement therapies are available to functionally compensate for α-galactosidase deficiency. Agalsidase α and β are both recombinant forms of the human α-galactosidase A enzyme and both have the same amino acid sequence as the native enzyme. Agalsidase α and β differ in the structures of their oligosaccharide side chains.In Fabry disease patients, 88% percent of patients develop IgG antibodies towards the injected recombinant enzyme, as it is foreign to their immune system. One suggested approach to solving this problem involves converting the paralogous enzyme α-NAGAL (NAGA) into one that has with α-GAL activity. Because patients still have a functional NAGA gene, their immune system will not produce NAGA antibodies.
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.