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Literature summary for 3.2.1.22 extracted from

  • Ishii, S.; Chang, H.H.; Kawasaki, K.; Yasuda, K.; Wu, H.L.; Garman, S.C.; Fan, J.Q.
    Mutant alpha-galactosidase A enzymes identified in Fabry disease patients with residual enzyme activity: biochemical characterization and restoration of normal intracellular processing by 1-deoxygalactonojirimycin (2007), Biochem. J., 406, 285-295.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.2.1.22

Activating Compound

Activating Compound Comment Organism Structure
1-deoxygalactonojirimycin 3.8fold increase at 0.02 mM after 5 days Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
mutants expressed in COS-7 cells Homo sapiens

Protein Variants

Protein Variants Comment Organism
A156V reduced activity Homo sapiens
A20P reduced activity Homo sapiens
A97V reduced activity Homo sapiens
E59K reduced activity Homo sapiens
E66Q reduced activity Homo sapiens
F113L reduced activity Homo sapiens
I91T reduced activity Homo sapiens
L166V reduced activity Homo sapiens
M296I reduced activity Homo sapiens
M296V reduced activity Homo sapiens
M72V reduced activity Homo sapiens
N215S reduced activity Homo sapiens
Q279E reduced activity Homo sapiens
R112H reduced activity Homo sapiens
R301Q reduced activity Homo sapiens
R356W reduced activity Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
1.8
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme Q279E, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
1.9
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme R301Q, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
2
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme R356W, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
2.4
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme M296V, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
2.6
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme L166V, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
2.7
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme M296I, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
2.8
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme A20P, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
2.8
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme N215S, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
2.8
-
 4-methylumbelliferyl-alpha-D-galactoside wild type enzyme, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
3.1
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme E66Q, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
3.2
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme M72V, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
3.4
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme A156V, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
3.4
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme I91T, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
3.6
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme F113L, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
3.7
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme A97V, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
5.8
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme R112H, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens
16.3
-
 4-methylumbelliferyl-alpha-D-galactoside mutant enzyme E59K, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
46000
-
 mature lysosomal enzyme form, SDS-PAGE Homo sapiens
50000
-
 ER and Golgi enzyme forms, SDS-PAGE Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Purification (Commentary)

Purification (Comment) Organism
-
 Homo sapiens

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
 Homo sapiens
-
lymphoblast
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4-methylumbelliferyl-alpha-D-galactoside + H2O
-
 Homo sapiens 4-methylumbelliferol + alpha-D-galactose
-
 ?

Synonyms

Synonyms Comment Organism
alpha-Gal A
-
 Homo sapiens
alpha-galactosidase A
-
 Homo sapiens

Temperature Stability [°C]

Temperature Stability Minimum [°C] Temperature Stability Maximum [°C] Comment Organism
37
-
 retains more than half of its activity after pre-incubation at pH 7.5 and 37°C for 90 min Homo sapiens

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
4 4.5
-
 Homo sapiens

pH Range

pH Minimum pH Maximum Comment Organism
3 7.5 wild type enzyme Homo sapiens
3.5 6.5 mutant enzymes Homo sapiens

pH Stability

pH Stability pH Stability Maximum Comment Organism
7.5
-
 retains more than half of its activity after pre-incubation at pH 7.5 and 37°C for 90 min Homo sapiens