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Literature summary extracted from

  • Chen, Y.; Jin, M.; Egborge, T.; Coppola, G.; Andre, J.; Calhoun, D.H.
    Expression and characterization of glycosylated and catalytically active recombinant human alpha-galactosidase A produced in Pichia pastoris (2000), Protein Expr. Purif., 20, 472-484.
    View publication on PubMed

Application

EC Number Application Comment Organism
3.2.1.22 medicine Fabry disease is a X-linked inborn error of glycolipid metabolism caused by deficiency of the lysosomal enzyme alpha-galactosidase A. An improved method of production of recombinant alpha-galactosidase A for use in humans is needed in order to develop new approaches for enzyme therapy. Purified recombinant enzyme is taken up by fibroblasts derived from fabry disease patients and normal enzyme levels can be restored under theses conditions Homo sapiens

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.2.1.22 expression in Pichia pastoris. Purified recombinant enzyme is taken up by fibroblasts dericed from fabry disease patients and normal enzyme levels can be restored under theses conditions Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
3.2.1.22 additional information Homo sapiens Fabry disease is a X-linked inborn error of glycolipid metabolism caused by deficiency of the lysosomal enzyme alpha-galactosidase A. This enzyme is responsible for the hydrolysis of terminal alpha-galactosidase linkages in various glycolipids ?
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
3.2.1.22 Homo sapiens
-
-
-

Posttranslational Modification

EC Number Posttranslational Modification Comment Organism
3.2.1.22 glycoprotein N-linked high-mannose structures rather than complex carbohydrates are present in the recombinant enzyme Homo sapiens

Purification (Commentary)

EC Number Purification (Comment) Organism
3.2.1.22
-
 Homo sapiens

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
3.2.1.22 74.62
-
 purified recombinant enzyme Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.2.1.22 4-methylumbelliferyl alpha-D-galactoside + H2O
-
 Homo sapiens 4-methylumbelliferone + alpha-D-galactose
-
 ?
3.2.1.22 additional information Fabry disease is a X-linked inborn error of glycolipid metabolism caused by deficiency of the lysosomal enzyme alpha-galactosidase A. This enzyme is responsible for the hydrolysis of terminal alpha-galactosidase linkages in various glycolipids Homo sapiens ?
-
 ?