Protein Variants | Comment | Organism |
---|---|---|
betaAsp452Asn/betaLeu453Arg | the mutant enzyme of HexB exhibits more than 30fold increase in its ability to hydrolyze a 6-sulfated substrate and is able to hydrolyze GM2 ganglioside when the GM2 activator protein is replaced by sodium taurocholate | Homo sapiens |
R424Q | the mutant form of HexA shows about 3fold increase in its Km-value for the complex of GM2 activator protein and GM2 ganglioside | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.046 | - |
complex of GM2 activator protein and GM2 ganglioside | wild-type enzyme | Homo sapiens | |
0.12 | - |
complex of GM2 activator protein and GM2 ganglioside | mutant enzyme R424Q | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | Homo sapiens | deficiency of either of the two subunits results in severe neurological disease due to the storage of GM2 gangliodide - Tay-Sachs disease, alpha deficiency, and Sandhoff disease, beta deficiency | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
placenta | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
complex of GM2 activator protein and GM2 ganglioside + H2O | - |
Homo sapiens | ? | - |
? | |
GM2 ganglioside + H2O | - |
Homo sapiens | ? | - |
? | |
additional information | deficiency of either of the two subunits results in severe neurological disease due to the storage of GM2 gangliodide - Tay-Sachs disease, alpha deficiency, and Sandhoff disease, beta deficiency | Homo sapiens | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
beta-hexosaminidase A | - |
Homo sapiens |