Entity Details

Primary name ASPG_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP20933
EntryNameASPG_HUMAN
FullNameN(4)-(beta-N-acetylglucosaminyl)-L-asparaginase
TaxID9606
Evidenceevidence at protein level
Length346
SequenceStatuscomplete
DateCreated1991-02-01
DateModified2021-06-02

Ontological Relatives

GenesAGA

GO terms

Show/Hide Table
GOName
GO:0003948 N4-(beta-N-acetylglucosaminyl)-L-asparaginase activity
GO:0005576 extracellular region
GO:0005615 extracellular space
GO:0005737 cytoplasm
GO:0005764 lysosome
GO:0005783 endoplasmic reticulum
GO:0006517 protein deglycosylation
GO:0008233 peptidase activity
GO:0035578 azurophil granule lumen
GO:0043312 neutrophil degranulation
GO:0043621 protein self-association

Subcellular Location

Show/Hide Table
Subcellular Location
Lysosome

Domains

Show/Hide Table
DomainNameCategoryType
IPR000246 Peptidase T2, asparaginase 2FamilyFamily
IPR029055 Nucleophile aminohydrolases, N-terminalFamilyHomologous superfamily

Diseases

Show/Hide Table
Disease IDSourceNameDescription
208400 OMIMAspartylglucosaminuria (AGU)An inborn lysosomal storage disease causing excess accumulation of glycoasparagine in the body tissues and its increased excretion in urine. Clinical features include mild to severe mental retardation manifesting from the age of two, coarse facial features and mild connective tissue abnormalities. The disease is caused by variants affecting the gene represented in this entry.