Entity Details

Primary name FUCO_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP04066
EntryNameFUCO_HUMAN
FullNameTissue alpha-L-fucosidase
TaxID9606
Evidenceevidence at protein level
Length466
SequenceStatuscomplete
DateCreated1986-11-01
DateModified2021-06-02

Ontological Relatives

GenesFUCA1

GO terms

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GOName
GO:0004560 alpha-L-fucosidase activity
GO:0005576 extracellular region
GO:0005737 cytoplasm
GO:0005764 lysosome
GO:0006004 fucose metabolic process
GO:0006027 glycosaminoglycan catabolic process
GO:0016139 glycoside catabolic process
GO:0019377 glycolipid catabolic process
GO:0035578 azurophil granule lumen
GO:0043202 lysosomal lumen
GO:0043312 neutrophil degranulation
GO:0070062 extracellular exosome

Subcellular Location

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Subcellular Location
Lysosome

Domains

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DomainNameCategoryType
IPR000933 Glycoside hydrolase, family 29FamilyFamily
IPR013780 Glycosyl hydrolase, all-betaFamilyHomologous superfamily
IPR016286 Alpha-L-fucosidase, metazoa-typeFamilyFamily
IPR017853 Glycoside hydrolase superfamilyFamilyHomologous superfamily
IPR018526 Glycoside hydrolase, family 29, conserved siteSiteConserved site
IPR028755 Tissue alpha-L-fucosidaseFamilyFamily
IPR031919 Alpha-L-fucosidase, C-terminalDomainDomain

Diseases

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Disease IDSourceNameDescription
230000 OMIMFucosidosis (FUCA1D)An autosomal recessive lysosomal storage disease characterized by accumulation of fucose-containing glycolipids and glycoproteins in various tissues. Clinical signs include facial dysmorphism, dysostosis multiplex, moderate hepatomegaly, severe intellectual deficit, deafness, and according to age, angiokeratomas. The disease is caused by variants affecting the gene represented in this entry.