Names & Taxonomy

Uniprot ID:
Q9UK23
Entry Name:
NAGPA_HUMAN
Status:
reviewed
Protein Names:
N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase (EC 3.1.4.45) (Mannose 6-phosphate-uncovering enzyme) (Phosphodiester alpha-GlcNAcase)
Gene Names:
NAGPA
Gene Names Primary:
NAGPA
Organism:
Homo sapiens (Human)

Structure

Length:
515
Sequence:
MATSTGRWLLLRLALFGFLWEASGGLDSGASRDDDLLLPYPRARARLPRDCTRVRAGNREHESWPPPPATPGAGGLAVRTFVSHFRDRAVAGHLTRAVEPLRTFSVLEPGGPGGCAARRRATVEETARAADCRVAQNGGFFRMNSGECLGNVVSDERRVSSSGGLQNAQFGIRRDGTLVTGYLSEEEVLDTENPFVQLLSGVVWLIRNGSIYINESQATECDETQETGSFSKFVNVISARTAIGHDRKGQLVLFHADGQTEQRGINLWEMAEFLLKQDVVNAINLDGGGSATFVLNGTLASYPSDHCQDNMWRCPRQVSTVVCVHEPRCQPPDCHGHGTCVDGHCQCTGHFWRGPGCDELDCGPSNCSQHGLCTETGCRCDAGWTGSNCSEECPLGWHGPGCQRPCKCEHHCPCDPKTGNCSVSRVKQCLQPPEATLRAGELSFFTRTAWLALTLALAFLLLISTAANLSLLLSRAERNRRLHGDYAYHPLQEMNGEPLAAEKEQPGGAHNPFKD
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Golgi apparatus, Golgi stack membrane

Function

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. Also hydrolyzes UDP-GlcNAc, a sugar donor for Golgi N-acetylglucosaminyltransferases.
Pathway:
Protein modification; protein glycosylation.
Catalytic Activity:
Glycoprotein N-acetyl-D-glucosaminyl-phospho-D-mannose + H(2)O = N-acetyl-D-glucosamine + glycoprotein phospho-D-mannose.
Cross Reference Drug Bank:
DB00141
Gene Ontology Go:
Golgi cisterna membrane
integral component of membrane
N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase activity
carbohydrate metabolic process
cellular protein modification process
lysosome organization
protein glycosylation
protein targeting to lysosome
Gene Ontology Biological Process:
carbohydrate metabolic process
cellular protein modification process
lysosome organization
protein glycosylation
protein targeting to lysosome
Gene Ontology Molecular Function:
N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase activity
Gene Ontology Cellular Component:
Golgi cisterna membrane
integral component of membrane
Keywords:
Alternative splicing
Complete proteome
Direct protein sequencing
Disulfide bond
EGF-like domain
Glycoprotein
Golgi apparatus
Hydrolase
Membrane
Polymorphism
Reference proteome
Signal
Transmembrane
Transmembrane helix
Zymogen

Publication

PubMed ID:
10551838 14702039 15616553 15489334 12058031 20147709 23572527 25944712