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Literature summary for 3.2.1.106 extracted from

  • McVie-Wylie, A.J.; Lee, K.L.; Qiu, H.; Jin, X.; Do, H.; Gotschall, R.; Thurberg, B.L.; Rogers, C.; Raben, N.; OCallaghan, M.; Canfield, W.; Andrews, L.; McPherson, J.M.; Mattaliano, R.J.
    Biochemical and pharmacological characterization of different recombinant acid alpha-glucosidase preparations evaluated for the treatment of Pompe disease (2008), Mol. Genet. Metab., 94, 448-455.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.2.1.106

Application

Application Comment Organism
medicine treatment of Pompe Disease Cricetulus griseus

Protein Variants

Protein Variants Comment Organism
additional information HP-GAA, carbohydraded engineered form, highest relative affinity to the soluble cation independent mannose-6-phosphate receptor Cricetulus griseus

Localization

Localization Comment Organism GeneOntology No. Textmining
lysosome
-
 Cricetulus griseus 5764
-

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
110000
-
 SDS-PAGE Cricetulus griseus

Organism

Organism UniProt Comment Textmining
Cricetulus griseus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
CHO cell
-
 Cricetulus griseus
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
4.2
-
 tgGAA Cricetulus griseus
4.3
-
 isoenzyme CHO-GAA Cricetulus griseus
4.5
-
 isoenzyme HP-GAA Cricetulus griseus

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4-methylumbelliferyl-alpha-D-glucoside pH 4.0, 60 min, 37°C Cricetulus griseus 4-methylumbelliferone + alpha-D-glucose
-
 ?
4-nitrophenyl D-alpha-glucopyranoside + H2O pH 4.3 Cricetulus griseus 4-nitrophenol + D-glucose
-
 ?

Synonyms

Synonyms Comment Organism
acid alpha-glucosidase
-
 Cricetulus griseus
CHO-GAA
-
 Cricetulus griseus
GAA
-
 Cricetulus griseus
HP-GAA
-
 Cricetulus griseus
rhGAA
-
 Cricetulus griseus
tgGAA
-
 Cricetulus griseus

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
37
-
 pH 4.0-4.3, 60 min Cricetulus griseus

pH Range

pH Minimum pH Maximum Comment Organism
4 4.3 37°C, 60 min Cricetulus griseus

pI Value

Organism Comment pI Value Maximum pI Value
Cricetulus griseus CHO-GAA, IEF-Gel 4.6 4
Cricetulus griseus tgGAA, IEF-Gel 4.6 4.2
Cricetulus griseus HP-GAA, IEF-Gel 4.9 4.6