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Literature summary for 3.2.1.20 extracted from

  • Zhang, H.; Kallwass, H.; Young, S.P.; Carr, C.; Dai, J.; Kishnani, P.S.; Millington, D.S.; Keutzer, J.; Chen, Y.T.; Bali, D.
    Comparison of maltose and acarbose as inhibitors of maltase-glucoamylase activity in assaying acid alpha-glucosidase activity in dried blood spots for the diagnosis of infantile Pompe disease (2006), Genet. Med., 8, 302-306.
    View publication on PubMed

General Stability

General Stability Organism
neutral alpha-glucosidase is less stable than acid alpha-glucosidase Homo sapiens

Inhibitors

Inhibitors Comment Organism Structure
acarbose
-
Homo sapiens
maltose
-
Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens a severe form of enzyme deficiency causes the infantile Pompe disease ?
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
patients with infantile Pompe disease
-

Source Tissue

Source Tissue Comment Organism Textmining
blood dried blood spot specimen from patients with infantile Pompe disease Homo sapiens
-
fibroblast
-
Homo sapiens
-
skin fibroblasts Homo sapiens
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
additional information
-
-
Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4-methylumbelliferyl-alpha-D-glucopyranoside + H2O
-
Homo sapiens 4-methylumbelliferol + alpha-D-glucose
-
?
additional information a severe form of enzyme deficiency causes the infantile Pompe disease Homo sapiens ?
-
?
additional information development and evaluation of an assay method for enzyme detection in dried blood spot specimen from patients with infantile Pompe disease, overview Homo sapiens ?
-
?

Synonyms

Synonyms Comment Organism
acid alpha-glucosidase
-
Homo sapiens
maltase-glucoamylase
-
Homo sapiens
neutral alpha-glucosidase
-
Homo sapiens

Temperature Optimum [°C]

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

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
3.8
-
acid alpha-glucosidase assay at Homo sapiens
7
-
neutral alpha-glucosidase assay at Homo sapiens