N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase
External IDs OMIM607985 MGI1351598 HomoloGene8466 GeneCards: NAGPA Gene
EC number
RNA expression pattern
PBB GE NAGPA 205090 s at tn.png
More reference expression data
Species Human Mouse
Entrez 51172 27426
Ensembl ENSG00000103174 ENSMUSG00000023143
UniProt Q9UK23 Q3TDF9
RefSeq (mRNA) NM_016256 NM_013796
RefSeq (protein) NP_057340 NP_038824.2
Location (UCSC) Chr 16:
5.07 – 5.08 Mb
Chr 16:
5.2 – 5.2 Mb
PubMed search [1] [2]

N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase is an enzyme that in humans is encoded by the NAGPA gene.[1][2][3]

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 C-terminus contains multiple signals for trafficking it between lysosomes, the plasma membrane, and trans-Golgi network.[3]


  1. ^ Kornfeld R, Bao M, Brewer K, Noll C, Canfield W (Jan 2000). "Molecular cloning and functional expression of two splice forms of human N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase". J Biol Chem 274 (46): 32778–85. doi:10.1074/jbc.274.46.32778. PMID 10551838. 
  2. ^ Do H, Lee WS, Ghosh P, Hollowell T, Canfield W, Kornfeld S (Aug 2002). "Human mannose 6-phosphate-uncovering enzyme is synthesized as a proenzyme that is activated by the endoprotease furin". J Biol Chem 277 (33): 29737–44. doi:10.1074/jbc.M202369200. PMID 12058031. 
  3. ^ a b "Entrez Gene: NAGPA N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase". 

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