Cloned (Comment) | Organism |
---|---|
cloned to an improved baculovirus vector and expressed in insect cells at optimized growth conditions, the purified enzyme is taken up by Fabry fibroblasts in culture resulting in normal enzyme levels | Homo sapiens |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
46620 | - |
laser desorption time-off-flight analysis | Homo sapiens |
46850 | - |
electrospray mass spectrometry | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
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 | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Posttranslational Modification | Comment | Organism |
---|---|---|
glycoprotein | carbohydrate accounts for about 10% of the mass of the glycoprotein. The carbohydrate moiety is composed of fucose, galactose, mannose, mannose-6-phosphate and N-acetylglucosamine. The human enzyme produced in insect cells has a higher fraction of complex structures, has a smaller fraction of high-mannose structures and contains a similar relative mass of carbohydrate compared to the enzyme produced in humans or vertebrate cell culture | Homo sapiens |
Purification (Comment) | Organism |
---|---|
recombinant enzyme | Homo sapiens |
Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
---|---|---|---|
0.052 | - |
- |
Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | Fabry disease is a X-linked inborn error of glycolipid metabolism caused by deficiency of the lysosomal enzyme alpha-galactosidase A | Homo sapiens | ? | - |
? |