InterPro domain: IPR016286

O-Glycosyl hydrolases family 29 ( GH29 ) encompasses alpha-L-fucosidases ( 3.2.1.51 ) [ 1 ], which is a lysosomal enzyme responsible for hydrolysing the alpha-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of the carbohydrate moieties of glycoproteins. 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.

Fucosylated glycoconjugates are involved in numerous biological events, making alpha-l-fucosidases, the enzymes responsible for their processing, critically important. Deficiency in alpha-l-fucosidase activity is associated with fucosidosis, a lysosomal storage disorder characterised by rapid neurodegeneration, resulting in severe mental and motor deterioration [ 2 ]. The enzyme is a hexamer and displays a two-domain fold, composed of a catalytic (beta/alpha)(8)-like domain and a C-terminal beta-sandwich domain [ 3 ].

Drosophila melanogaster spermatozoa contains an alpha-l-fucosidase that might be involved in fertilisation by interacting with alpha-l-fucose residues on the micropyle of the eggshell [ 3 ]. In human sperm, membrane-associated alpha-l-fucosidase is stable for extended periods of time, which is made possible by membrane domains and compartmentalisation. These help preserve protein integrity [ 4 ].

This entry represents a subgroup of alpha-L-fucosidases found in metazoa, fungi and bacteria.

1. Isolation and sequence analysis of a cDNA encoding rat liver alpha-L-fucosidase. Biochem. J. 264, 695-701
2. Crystal structure of Thermotoga maritima alpha-L-fucosidase. Insights into the catalytic mechanism and the molecular basis for fucosidosis. J. Biol. Chem. 279, 13119-28
3. Expression study of an alpha-l-fucosidase gene in the Drosophilidae family. Gene 420, 23-33
4. Stabilization of membrane-associated alpha-l-fucosidase by the human sperm equatorial segment. Int. J. Androl.