|PROSITE documentation PDOC00324 [for PROSITE entry PS00385]|
α-L-fucosidase (EC 126.96.36.199)  is a lysosomal enzyme responsible for hydrolyzing the α-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of the carbohydrate moieties of glycoproteins. Deficiency of α-L-fucosidase results in the lysosomal storage disease fucosidosis.
A cysteine residue is important for the activity of the enzyme. There is only one cysteine conserved between the sequence of mammalian α-L-fucosidase and that of the slime mold Dictyostelium discoideum. We have derived a pattern from the region around that conserved cysteine.Note:
May 2004 / Text revised.
PROSITE method (with tools and information) covered by this documentation:
|1||Authors||Fisher K.J., Aronson N.N. Jr.|
|Title||Isolation and sequence analysis of a cDNA encoding rat liver alpha-L-fucosidase.|
|Source||Biochem. J. 264:695-701(1989).|
|Title||A classification of glycosyl hydrolases based on amino acid sequence similarities.|
|Source||Biochem. J. 280:309-316(1991).|