Hydrolases are transported to lysosomes after binding to mannose 6-phosphate receptors in the trans-Golgi network. This gene encodes the enzyme that catalyzes the second step in the formation of the mannose 6-phosphate recognition marker on lysosomal hydrolases. Commonly known as 'uncovering enzyme' or UCE, this enzyme removes N-acetyl-D-glucosamine (GlcNAc) residues from GlcNAc-alpha-P-mannose moieties and thereby produces the recognition marker. This reaction most likely occurs in the trans-Golgi network. This enzyme functions as a homotetramer of two disulfide-linked homodimers. In addition to having an N-terminal signal peptide, the protein's SLCterminus contains multiple signals for trafficking it between lysosomes, the plasma membrane, and trans-Golgi network. [provided by RefSeq, Jul 2008]
Function:
Catalyzes the second step in the formation of the mannose 6-phosphate targeting signal on lysosomal enzyme oligosaccharides by removing GlcNAc residues from GlcNAc-alpha-P-mannose moieties, which are formed in the first step.
Subunit:
Homotetramer arranged as two disulfide-linked homodimers
Subcellular Location:
Golgi apparatus > Golgi stack membrane. Cis/medial Golgi.
Tissue Specificity:
Isoform 2 may be brain-specific.
Post-translational modifications:
The precursor is cleaved and activated in the trans-Golgi network by a furin endopeptidase.
Similarity:
Contains 1 EGF-like domain.
SWISS:
Q9UK23
Gene ID:
51172
Database links:
Entrez Gene: 515935 Cow
Entrez Gene: 98019 Dog
Entrez Gene: 100723215 Guinea pig
Entrez Gene: 100069983 Horse
Entrez Gene: 51172 Human
Entrez Gene: 27426 Mouse
Entrez Gene: 100512596 Pig
Entrez Gene: 72476 Rat
Omim: 607985 Human
SwissProt: P68827 Cow
SwissProt: Q9UK23 Human
SwissProt: Q8BJ48 Mouse
Unigene: 21334 Human
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