Tissue alpha-L-Fucosidase/FUCA1 Proteins and Enzymes

Alpha-L-fucosidase (EC 3.2.1.51) is a lysosomal enzyme involved in the degradation of fucose-containing glycoproteins and glycolipids (Occhiodoro et al., 1989 [PubMed 2803312]). At least 2 separate polymorphic alpha-L-fucosidases are recognized in man: that in tissues, FUCA1, which is deficient in fucosidosis (MIM 230000), and that in plasma, FUCA2 (MIM 136820).[supplied by OMIM]