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FLASH GENE
Symbol AGA contributors: npt/ - updated : 13-10-2006
HGNC name aspartylglucosaminidase
HGNC id 318
Corresponding disease
AGU aspartylglucosaminuria
Location 4q34.3      Physical location : 178.351.930 - 178.363.657
Synonym name
  • N4-(N-acetyl-beta-glucosaminyl)-L-asparagine amidase
  • glycosylasparaginase
  • aspartylglucosylamine deaspartylase
  • Synonym symbol(s) GA, ASRG
    EC.number 3.5.1.26
    DNA
    TYPE functioning gene
    STRUCTURE 11.67 kb     9 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked Y status confirmed
    Physical map
    HMGB2 4q31 high-mobility group box 2 SAP30 4q34.1 sin3-associated polypeptide, 30kDa SCRG1 4q31-q32 sin3-associated polypeptide, 30kDa LOC391716 4 similar to 60S ribosomal protein L5 LOC389242 4 LOC389242 HAND2 4q33 heart and neural crest derivatives expressed 2 FLJ11539 4q34.1 hypothetical protein FLJ11539 MORF4 4q33-q34.1 mortality factor 4 LOC391717 4 similar to RAN protein LOC389243 4 LOC389243 FBXO8 4q34.1 F-box only protein 8 KIAA1712 4q34 KIAA1712 HPGD 4q34-q35 hydroxyprostaglandin dehydrogenase 15-(NAD) GLRA3 4q33-q34 glycine receptor, alpha 3 ADAM29 4q34 a disintegrin and metalloproteinase domain 29 LOC391718 4 similar to hypothetical protein MGC2776 GPM6A 4q34 glycoprotein M6A WDR17 4q34 WD repeat domain 17 TSARG2 4q34.2 testis and spermatogenesis cell related protein 2 ASB5 4q34.1 ankyrin repeat and SOCS box-containing 5 FLJ22649 4q34.2 hypothetical protein FLJ22649 similar to signal peptidase SPC22/23 VEGFC 4q34 vascular endothelial growth factor C FLJ10858 4q34.3 DNA glycosylase hFPG2 AGA 4q33-q35 aspartylglucosaminidase LOC285501 4q34.3 hypothetical protein LOC285501 LOC391719 4 similar to hypothetical protein LOC391720 4 similar to NADH dehydrogenase LOC389244 4 LOC389244 LOC132386 4q35.1 tumor-associated calcium signal transducer 1 pseudogene
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    9 - 2113 - 346 - -
    - - 2083 - 336 - -
    EXPRESSION
    Type widely
    constitutive of
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularvessel   moderately
    Digestiveintestinesmall intestine   
     pancreas exocrine   highly
    Lymphoid/Immunelymph node   moderately
    Nervousbrain    
    Reproductivefemale systemuteruscervix predominantly
     male systemtestis  moderately
    Respiratorylung   moderately
    Skin/Tegumentskin   highly
    Urinarybladder   highly
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Blood / Hematopoieticbone marrow   
    Connective    
    Epithelialabsorptive excretorydigestive epithelium  
    Epithelialbarrier/lininguroepithelium  
    Epithelialsecretoryglandularexocrine 
    Lymphoid    
    cells
    SystemCellPubmedSpeciesStageRna symbol
     fibroblast
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    physiological period pregnancy
    Text amniotic fluid cells
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • N-terminal signal peptide of 23 AAs is removed in the ER, and then dimerization and glycosylation of the two precursor peptides occur (Saito 2008)
  • conjugated GlycoP
    mono polymer heteromer , tetramer
    isoforms Precursor inactive AGA precursor consisting of 346 amino acid residues on endoplasmic reticulum (ER)-bound ribosomes (Saito 2008)
    HOMOLOGY
    interspecies ortholog to murine Aga
    Homologene
    FAMILY
  • Ntn-hydrolase family
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,endoplasmic reticulum ,rough,smooth
    intracellular,cytoplasm,organelle,Golgi
    intracellular,cytoplasm,organelle,endosome
    intracellular,cytoplasm,organelle,lysosome
    basic FUNCTION
  • involved in the catabolism of N-linked oligosaccharides of glycoproteins
  • cleaving asparagine from N-acetylglucosamines as one of the final steps in the lysosomal breakdown of glycoproteins
  • CELLULAR PROCESS protein, degradation
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism carbohydrate , other
    signaling
    catabolism of glycoproteins (protein deglycosylation)
    a component
  • heterotetramer of two alpha and two beta chains bound by disulfide bonds
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
    cell & other
    REGULATION
    Other targeted to the lysosome by M6P receptor mediated pathway
    ASSOCIATED DISORDERS
    corresponding disease(s) AGU
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS