Reactome | FUCA1 hydrolyses NGP:1,6-GlcNAc

FUCA1 hydrolyses NGP:1,6-GlcNAc

Stable Identifier

R-HSA-5693807

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lysosomal lumen

ReviewStatus

5/5

Locations in the PathwayBrowser

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Metabolism of proteins (Homo sapiens)

- Post-translational protein modification (Homo sapiens)
  - Asparagine N-linked glycosylation (Homo sapiens)
    - Transport to the Golgi and subsequent modification (Homo sapiens)
      - N-glycan antennae elongation in the medial/trans-Golgi (Homo sapiens)
        
        Reactions specific to the complex N-glycan synthesis pathway (Homo sapiens)
        
        FUCA1 hydrolyses NGP:1,6-GlcNAc (Homo sapiens)

General

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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).

Literature References

PubMed ID	Title	Journal	Year
10094192	Spectrum of mutations in fucosidosis Willems, PJ, Seo, HC, Coucke, P, Tonlorenzi, R, O'Brien, JS	Eur. J. Hum. Genet.	1999
17175120	Comparative and phylogenetic analysis of alpha-L-fucosidase genes Intra, J, Perotti, ME, Pavesi, G, Horner, D	Gene	2007