α-''N''-acetylgalactosaminidase () is a
glycoside hydrolase
In biochemistry, glycoside hydrolases (also called glycosidases or glycosyl hydrolases) are a class of enzymes which catalysis, catalyze the hydrolysis of glycosidic bonds in polysaccharide, complex sugars. They are extremely common enzymes, wi ...
from bacteria and animals, also known as nagalase.
The human gene that codes for this enzyme is
NAGA. Mutations in this gene and the deficiency in α-''N''-acetylgalactosaminidase activity have been identified as the cause of
Schindler disease.
Enzymes with this activity is useful for converting
type A blood to type O, giving it the name of A-zyme. Bacterial enzymes in the GH109 family, including the A-zyme from ''
Elizabethkingia meningoseptica
''Elizabethkingia meningoseptica'' is a Gram-negative, rod-shaped bacterium widely distributed in nature (e.g. fresh water, salt water, or soil). It may be normally present in fish and frogs; it may be isolated from chronic infectious states, as ...
'', runs this reaction efficiently.
Nagalase catalyzes the deglycosylation of the Gc protein also known as vitamin D3 binding protein rendering it incapable of being converted to the regulatory protein, Gc Macrophage Activating Factor, a protein involved in the activation of macrophages — in cases when many types of incompatible cells happen to be around, thus nagalase diminishes the body’s macrophage activating capacity, and elevated nagalase has been reported in systemic disorders including systemic lupus erythematosus. Elevated nagalase levels have also been found in the blood of children with autism and autism spectrum disorders. Nagalase accumulates in the serum of cancer patients and its activity correlates with tumor burden, aggressiveness and clinical disease progression.
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EC 3.2.1
Enzymes