Aspartylglucosaminidase

N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase is an enzyme that in humans is encoded by the AGA gene.^[5]

AliasesAGA, Aga, AW060726, AGU, ASRG, GA, aspartylglucosaminidase

External IDsOMIM: 613228; MGI: 104873; GeneCards: AGA

PDBOrtholog search: PDBe RCSB

Chr.Chromosome 4 (human)^[1]

Quick facts AGA, Identifiers ...

AGA
Identifiers
Aliases	AGA, Aga, AW060726, AGU, ASRG, GA, aspartylglucosaminidase
External IDs	OMIM: 613228; MGI: 104873; GeneCards: AGA
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 1APY, 1APZ
Enzyme activity EC # BRENDA ExPASy KEGG MetaCyc 3.5.1.26 ↗ ↗ ↗ ↗
Gene location (Human) Chr. Chromosome 4 (human)[1] Band 4q34.3 Start 177,430,774 bp[1] End 177,442,437 bp[1]
Gene location (Mouse) Chr. Chromosome 8 (mouse)[2] Band 8|8 B1.3 Start 53,964,762 bp[2] End 53,976,456 bp[2]
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in corpus epididymis gingival epithelium gonad skin of hip stromal cell of endometrium skin of thigh oral cavity islet of Langerhans parotid gland kidney tubule Top expressed in calvaria ascending aorta otolith organ utricle pituitary gland aortic valve epithelium of lens stroma of bone marrow right kidney lobe of prostate More reference expression data BioGPS More reference expression data
Gene ontology Molecular function protein self-association peptidase activity hydrolase activity N4-(beta-N-acetylglucosaminyl)-L-asparaginase activity Cellular component lysosome endoplasmic reticulum extracellular region extracellular space azurophil granule lumen cytoplasm Biological process protein maturation protein deglycosylation proteolysis neutrophil degranulation Sources:Amigo / QuickGO
Orthologs Databases NCBI: entry; OMA: entry Species Human Mouse Entrez 175 11593 Ensembl ENSG00000038002 ENSMUSG00000031521 UniProt P20933 Q64191 RefSeq (mRNA) NM_000027 NM_001171988 NM_001005847 NM_001205054 RefSeq (protein) NP_000018 NP_001165459 NP_001005847 NP_001191983 Location (UCSC) Chr 4: 177.43 – 177.44 Mb Chr 8: 53.96 – 53.98 Mb PubMed search [3] [4]
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Aspartylglucosaminidase is an amidohydrolase enzyme involved in the catabolism of N-linked oligosaccharides of glycoproteins. It cleaves asparagine from N-acetylglucosamines as one of the final steps in the lysosomal breakdown of glycoproteins. The lysosomal storage disease aspartylglycosaminuria is caused by a deficiency in the AGA enzyme.^[5]