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Literature summary for 3.2.1.106 extracted from

  • Zhu, Y.; Jiang, J.L.; Gumlaw, N.K.; Zhang, J.; Bercury, S.D.; Ziegler, R.J.; Lee, K.; Kudo, M.; Canfield, W.M.; Edmunds, T.; Jiang, C.; Mattaliano, R.J.; Cheng, S.H.
    Glycoengineered Acid alpha-Glucosidase With Improved Efficacy at Correcting the Metabolic Aberrations and Motor Function Deficits in a Mouse Model of Pompe Disease (2009), Mol. Ther., 17, 954-963.
    View publication on PubMedView publication on EuropePMC

Application

Application Comment Organism
medicine treatment of Pompe Disease Homo sapiens

Protein Variants

Protein Variants Comment Organism
D404N same mutant as D518N Homo sapiens
D518N same mutant as D404N Homo sapiens
additional information variant oxime-neo-rhGAA, content of mannose-6-phosphat residues increased, higher affinity for the cation-independent mannose 6-phosphate receptor Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
additional information
-
additional information 50% increased Km-Value of the modified enzyme Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
lysosome
-
Homo sapiens 5764
-

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Posttranslational Modification

Posttranslational Modification Comment Organism
additional information chemical conjucation of synthetic oligosaccharides bearing M6P residues, neo-rhGAA Homo sapiens

Purification (Commentary)

Purification (Comment) Organism
-
Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4-methylumbelliferyl-alpha-D-glucopyranoside + H2O 4°C, pH 6.25 Homo sapiens 4-methylumbelliferone + alpha-D-glucose
-
?

Synonyms

Synonyms Comment Organism
acid alpha-glucosidase
-
Homo sapiens
glycoengineered acid alpha-glucosidase
-
Homo sapiens
oxime-neo-rhGAA
-
Homo sapiens
rhGAA
-
Homo sapiens

Temperature Stability [°C]

Temperature Stability Minimum [°C] Temperature Stability Maximum [°C] Comment Organism
4
-
for 90 days, higher stability of the modified enzyme Homo sapiens
25
-
for 14 days, higher stability of the modified enzyme Homo sapiens