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Literature summary for 3.2.1.20 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

Cloned(Commentary)

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

Localization

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

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
110000
-
CHO cell-derived rhGAA, HP-GAA, and tgGAA, SDS-PAGE Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Purification (Commentary)

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

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4-nitrophenyl 2-deoxy-alpha-D-glucopyranoside + H2O
-
Homo sapiens 4-nitrophenol + 2-deoxy-alpha-D-glucose
-
?
glycogen + H2O
-
Homo sapiens alpha-D-glucose
-
?

Synonyms

Synonyms Comment Organism
acid alpha-glucosidase
-
Homo sapiens
alglucosidase alfa
-
Homo sapiens
GAA
-
Homo sapiens
myozyme
-
Homo sapiens
recombinant human GAA
-
Homo sapiens
rhGAA
-
Homo sapiens

Temperature Optimum [°C]

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

pI Value

Organism Comment pI Value Maximum pI Value
Homo sapiens for CHO cell-derived rhGAA 4.6 4
Homo sapiens for CHO cell-derived tgGAA 4.6 4.2
Homo sapiens for CHO cell-derived HP-GAA 4.9 4.6