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Literature summary 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

Application

EC Number Application Comment Organism
3.2.1.106 medicine treatment of Pompe Disease Cricetulus griseus

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.2.1.20 produced in CHO cells (CHO-GAA). rhGAA purified from the milk of transgenic Oryctolagus cuniculus Homo sapiens

Protein Variants

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

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
3.2.1.20 lysosome
-
 Homo sapiens 5764
-
3.2.1.106 lysosome
-
 Cricetulus griseus 5764
-

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
3.2.1.20 110000
-
 CHO cell-derived rhGAA, HP-GAA, and tgGAA, SDS-PAGE Homo sapiens
3.2.1.106 110000
-
 SDS-PAGE Cricetulus griseus

Organism

EC Number Organism UniProt Comment Textmining
3.2.1.20 Homo sapiens
-
-
-
3.2.1.106 Cricetulus griseus
-
-
-

Purification (Commentary)

EC Number Purification (Comment) Organism
3.2.1.20 rhGAA purified from the milk of transgenic Oryctolagus cuniculus Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.2.1.106 CHO cell
-
 Cricetulus griseus
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
3.2.1.106 4.2
-
 tgGAA Cricetulus griseus
3.2.1.106 4.3
-
 isoenzyme CHO-GAA Cricetulus griseus
3.2.1.106 4.5
-
 isoenzyme HP-GAA Cricetulus griseus

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.2.1.20 4-nitrophenyl 2-deoxy-alpha-D-glucopyranoside + H2O
-
 Homo sapiens 4-nitrophenol + 2-deoxy-alpha-D-glucose
-
 ?
3.2.1.20 glycogen + H2O
-
 Homo sapiens alpha-D-glucose
-
 ?
3.2.1.106 4-methylumbelliferyl-alpha-D-glucoside pH 4.0, 60 min, 37°C Cricetulus griseus 4-methylumbelliferone + alpha-D-glucose
-
 ?
3.2.1.106 4-nitrophenyl D-alpha-glucopyranoside + H2O pH 4.3 Cricetulus griseus 4-nitrophenol + D-glucose
-
 ?

Synonyms

EC Number Synonyms Comment Organism
3.2.1.20 acid alpha-glucosidase
-
 Homo sapiens
3.2.1.20 alglucosidase alfa
-
 Homo sapiens
3.2.1.20 GAA
-
 Homo sapiens
3.2.1.20 myozyme
-
 Homo sapiens
3.2.1.20 recombinant human GAA
-
 Homo sapiens
3.2.1.20 rhGAA
-
 Homo sapiens
3.2.1.106 acid alpha-glucosidase
-
 Cricetulus griseus
3.2.1.106 CHO-GAA
-
 Cricetulus griseus
3.2.1.106 GAA
-
 Cricetulus griseus
3.2.1.106 HP-GAA
-
 Cricetulus griseus
3.2.1.106 rhGAA
-
 Cricetulus griseus
3.2.1.106 tgGAA
-
 Cricetulus griseus

Temperature Optimum [°C]

EC Number Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
3.2.1.20 37
-
 assay at Homo sapiens
3.2.1.106 37
-
 pH 4.0-4.3, 60 min Cricetulus griseus

pH Range

EC Number pH Minimum pH Maximum Comment Organism
3.2.1.106 4 4.3 37°C, 60 min Cricetulus griseus

pI Value

EC Number Organism Comment pI Value Maximum pI Value
3.2.1.20 Homo sapiens for CHO cell-derived rhGAA 4.6 4
3.2.1.20 Homo sapiens for CHO cell-derived tgGAA 4.6 4.2
3.2.1.20 Homo sapiens for CHO cell-derived HP-GAA 4.9 4.6
3.2.1.106 Cricetulus griseus CHO-GAA, IEF-Gel 4.6 4
3.2.1.106 Cricetulus griseus tgGAA, IEF-Gel 4.6 4.2
3.2.1.106 Cricetulus griseus HP-GAA, IEF-Gel 4.9 4.6