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Literature summary for 3.5.1.26 extracted from

  • Saarela, J.; Oinonen, C.; Jalanko, A.; Rouvinen, J.; Peltonen, L.
    Autoproteolytic activation of human aspartylglucosaminidase (2004), Biochem. J., 378, 363-371.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.5.1.26

Activating Compound

Activating Compound Comment Organism Structure
additional information intramolecular autoproteolytic activation with Gly-258 playing an important structural role, molecular activation mechanism Homo sapiens

Application

Application Comment Organism
medicine deficient AGA activity results in a lysosomal storage disease, aspartylglucosaminuria, resulting in progressive neurodegeneration, most of disease-causing mutations lead to defective molecular maturation of AGA Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
expression in COS-1 cells and in BHK-21 cells Homo sapiens

Protein Variants

Protein Variants Comment Organism
D200A 87% of wild-type activity, study of folding, transport and catalytic kinetics of mutant AGA Homo sapiens
D201A 93% of wild-type activity, study of folding, transport and catalytic kinetics of mutant AGA Homo sapiens
D70A 44% of wild-type activity, study of folding, transport and catalytic kinetics of mutant AGA Homo sapiens
G226A inactive mutant, study of folding, transport and catalytic kinetics of mutant AGA Homo sapiens
G258A inactive mutant, study of folding, transport and catalytic kinetics of mutant AGA Homo sapiens
K230A 86% of wild-type activity, study of folding, transport and catalytic kinetics of mutant AGA Homo sapiens
additional information aspartylglucosaminuria-causing mutations, most of them lead to defective molecular maturation of AGA, effects of targeted amino acid substitutions on the activation process of AGA Homo sapiens
N225A 45% of wild-type activity, study of folding, transport and catalytic kinetics of mutant AGA Homo sapiens
S238A 40% of wild-type activity, study of folding, transport and catalytic kinetics of mutant AGA Homo sapiens
T33A 48% of wild-type activity, study of folding, transport and catalytic kinetics of mutant AGA Homo sapiens
T33S same activity as wild-type AGA Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
additional information
-
 additional information
-
 Homo sapiens
0.143
-
 2-acetamido-1-beta-(L-aspartamido)-1,2-dideoxy-D-glucose pH 7, 37°C, purified wild-type AGA Homo sapiens
0.444
-
 2-acetamido-1-beta-(L-aspartamido)-1,2-dideoxy-D-glucose pH 7, 37°C, wild-type AGA Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
endoplasmic reticulum AGA maturation, activation cleavage of the dimerized AGA precursors into the N-terminal alpha- and the C-terminal beta-subunits takes place in the endoplasmic reticulum Homo sapiens 5783
-
lysosome enzymic catalysis takes place in lysosomes Homo sapiens 5764
-

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
44000
-
 1 * 44000, monomeric AGA precursor, SDS-PAGE Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens P20933
-
-

Posttranslational Modification

Posttranslational Modification Comment Organism
proteolytic modification intramolecular autoproteolytic activation with Gly-258 playing an important structural role, molecular activation mechanism, dimerization and correct folding of the AGA precursor, activation cleavage of the dimerized AGA precursors into the N-terminal alpha- and the C-terminal beta-subunits takes place in the endoplasmic reticulum and results in the tetrameric, enzymically active (alpha,beta)2 molecule Homo sapiens

Purification (Commentary)

Purification (Comment) Organism
-
 Homo sapiens

Reaction

Reaction Comment Organism Reaction ID
N4-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate mechanism Homo sapiens
a

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2-acetamido-1-beta-(L-aspartamido)-1,2-dideoxy-D-glucose + H2O
-
 Homo sapiens ?
-
 ?
N4-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O Thr-206 is the N-terminal nucleophile that acts as catalytic residue, Thr-206 is stabilized by hydrogen bonds from Ser-72 and Thr-224, enzyme structure Homo sapiens N-acetyl-beta-D-glucosaminylamine + L-aspartate
-
 ?

Subunits

Subunits Comment Organism
monomer 1 * 44000, monomeric AGA precursor, SDS-PAGE Homo sapiens
tetramer (alpha,beta)2, enzymically active AGA Homo sapiens

Synonyms

Synonyms Comment Organism
More belongs to the N-terminal nucleophile hydrolase superfamily Homo sapiens

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
37
-
 assay at Homo sapiens

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
7
-
 assay at Homo sapiens