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aspartylglucosaminidase (N-aspartyl-beta-glucosaminidase, N4-(beta-N-acetylglucosaminyl)-L-asparaginase, glycosylasparaginase, [contains: glycosylasparaginase alpha chain; glycosylasparaginase beta chain], AGA)
Function:
- cleaves the GlcNAc-Asn bond which joins oligosaccharides to the peptide of asparagine-linked glycoproteins
N(4)-(beta-N-acetyl-D-glucosaminyl)-L- asparagine + H2O
N-acetyl-beta-D-glucosaminylamine + L-aspartate
Structure:
- heterotetramer of two alpha & two beta chains
- belongs to the asparaginase 2 family
Compartment: Lysosome
Pathology:
- defects in AGA are the cause of aspartylglucosaminuria
Laboratory:
- aspartylglucosaminidase in fibroblasts
- aspartylglucosaminidase in leukocytes
Related
AGA gene mutation
General
amidohydrolase
lysosomal hydrolase
Properties
SIZE: entity length = 346 aa
MW = 37 kD
COMPARTMENT: lysosome
MOTIF: signal sequence {1-23}
N-glycosylation site {N38}
cysteine residue {C64}
MODIFICATION: cysteine residue {C69}
cysteine residue {C69}
MODIFICATION: cysteine residue {C64}
cysteine residue {C163}
MODIFICATION: cysteine residue {C179}
cysteine residue {C179}
MODIFICATION: cysteine residue {C163}
threonine residue {T206}
cysteine residue {C286}
MODIFICATION: cysteine residue {C306}
cysteine residue {C306}
MODIFICATION: cysteine residue {C286}
N-glycosylation site {N308}
cysteine residue {C317}
MODIFICATION: cysteine residue {C345}
cysteine residue {C345}
MODIFICATION: cysteine residue {C317}
Database Correlations
OMIM 208400
UniProt P20933
Pfam PF01112
Entrez Gene 175
ENZYME 3.5.1.26
References
- OMIM :accession 208400
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=AGA
- UniProt :accession P20933