Alpha-L-fucosidase is responsible for hydrolysing the alpha-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of the carbohydrate moieties of glycoproteins. In humans, this enzyme is encoded by two genes: FUCA1, which codes the tissue enzyme, and FUCA2, which leads to plasma alpha-L-fucosidase []. The significance of tissue alpha-L-fucosidase in metabolism is shown in the genetic neurovisceral storage disease fucosidosis, caused by a defect of the FUCA1 gene [, ].This entry represents the tissue alpha-L-fucosidase (FUCA1).
