Alpha-L-fucosidase is responsible for hydrolyzing the alpha-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of the carbohydrate moieties of glycoproteins. an alpha-L-fucoside + H2O = an alcohol + L-fucose a neolactoside IV(2)-alpha-Fuc-nLc4Cer(d18:1(4E)) + H2O = a neolactoside nLc4Cer(d18:1(4E)) + L-fucose a neolactoside IV(2)-alpha-Fuc-nLc4Cer(d18:0) + H2O = a neolactoside nLc4Cer(d18:0) + L-fucose Homotetramer. There are two common alleles of FUCA1; FUCA1*1; also known as Fu1; has Arg-281 and FUCA1*2; also known as Fu2; has Gln-281. The disease is caused by variants affecting the gene represented in this entry. Belongs to the glycosyl hydrolase 29 family. Truncated N-terminus. Truncated N-terminus. Truncated N-terminus. Truncated N-terminus. Translation of X01390 sequence produces a larger peptide than that shown in CAA25646.1.