α-N-acetylgalactosaminidase (EC 3.2.1.49) is a glycoside hydrolase from bacteria and animals, also known as nagalase.
Mutations in this gene and the deficiency in α-N-acetylgalactosaminidase activity have been identified as the cause of Schindler disease.
Bacterial enzymes in the GH109 family, including the A-zyme from Elizabethkingia meningoseptica, runs this reaction efficiently.
[2] 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.
[3] Nagalase accumulates in the serum of cancer patients and its activity correlates with tumor burden, aggressiveness and clinical disease progression.