Glycosylasparaginase

Function

Glycosylasparaginase or aspartylglucosaminidase (AGA) hydrolyzes a number of β-aspartyl amides including asparagine. AGA contains α and β subunits which derive from a precursor (PAGA) which is cleaved post-translationally. Mammalian AGA substrate is the protein-sugar bond of Asn-GlcNac.

Disease

AGA deficiency is the cause of the human lysosomal disease aspartylglycosaminuria.

Relevance

Structural highlights

3D structures of glycosylasparaginase

Updated on 16-June-2014

AGA α+β subunits

1ayy, 2gaw – EmAGA α+β subunits – Elizabethkingia meningoseptica

1apy – hAGA + Nag + β-mannose - human
1apz – hAGA + Nag + β-mannose + Asp
2gl9 – EmAGA α+β (mutant) subunits + Nag + Asn<br /

AGA precursor

2gac - EmPAGA (mutant)

9gaa, 1p4k – EmPAGA (mutant)
9gac, 9gaf, 1p4v, 3ljq – EmPAGA (mutant) + glycine

References