Aspartylglucosaminidase

AGA
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	1APY, 1APZ
Identifiers
Aliases	AGA, Aga, AW060726, AGU, ASRG, GA, aspartylglucosaminidase
External IDs	OMIM: 613228; MGI: 104873; HomoloGene: 13; GeneCards: AGA; OMA:AGA - orthologs
Gene location (Human)
Chr.	Chromosome 4 (human)
End	177,442,437 bp
Gene location (Mouse)
Chr.	Chromosome 8 (mouse)
End	53,976,456 bp
RNA expression pattern
	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
	; ; ; ;
	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
	175
	11593
	ENSG00000038002
	ENSMUSG00000031521
	P20933
	Q64191
	NM_000027; NM_001171988
	NM_001005847; NM_001205054
	NP_000018; NP_001165459
	NP_001005847; NP_001191983
	Wikidata
View/Edit Human	View/Edit Mouse

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

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.