Reaction: FUCA1 hydrolyses NGP:1,6-GlcNAc
Tissue alpha-L-fucosidase (FUCA1) is a lysosomal enzyme that removes terminal L-fucose residues from the oligosaccharide chains of N-glycoproteins (NGPs). In humans, FUCA1 encodes the tissue enzyme, whilst FUCA2 encodes plasma alpha-L-fucosidase (Intra et al. 2007). Defects in FUCA1 can cause fucosidosis (FUCA1D; MIM:230000), a rare lysosomal storage disorder characterised by progressive psychomotor deterioration, angiokeratoma and growth retardation (Willems et al. 1999).
Reaction - small molecule participants:
NGP [lysosomal lumen]
L-fucose [lysosomal lumen]
H2O [lysosomal lumen]
Reactome.org reaction link: R-HSA-5693807
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Reaction input - small molecules:
water
Reaction output - small molecules:
N-glycan
L-fucopyranose
Reactome.org link: R-HSA-5693807