Application | Comment | Organism |
---|---|---|
medicine | natural mutant P275L from a patient with recessive congenital methemoglobinemia shows significant decrease in the affinity toward the physiological reducing substrate, NADH, without affecting the activity | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
P275L | natural mutant from a patient with recessive congenital methemoglobinemia. Significant decrease in the affinity toward the physiological reducing substrate, NADH, without affecting the activity | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.004 | - |
ferricytochrome b5 | wild-type, 25°C, pH 7.0 | Homo sapiens | |
0.006 | - |
ferricyanide | mutant P275L, 25°C, pH 7.0 | Homo sapiens | |
0.006 | - |
NADH | mutant P275L, cosubstrate ferricyanide, 25°C, pH 7.0 | Homo sapiens | |
0.007 | - |
ferricyanide | wild-type, 25°C, pH 7.0 | Homo sapiens | |
0.012 | - |
ferricytochrome b5 | mutant P275L, 25°C, pH 7.0 | Homo sapiens | |
2.623 | - |
NADH | wild-type, cosubstrate ferricyanide, 25°C, pH 7.0 | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | P00387 | sequence of natural mutant gene from a patient with recessive congenital methemoglobinemia; patients with recessive congenital methemoglobinemia | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
2 ferricyanide + NADH | - |
Homo sapiens | 2 ferrocyanide + NAD+ + H+ | - |
? | |
2 ferricytochrome b5 + NADH | - |
Homo sapiens | 2 ferrocytochrome b5 + NAD+ + H+ | - |
? |
Turnover Number Minimum [1/s] | Turnover Number Maximum [1/s] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.733 | - |
NADH | wild-type, cosubstrate ferricyanide, 25°C, pH 7.0 | Homo sapiens | |
0.8 | - |
NADH | mutant P275L, cosubstrate ferricyanide, 25°C, pH 7.0 | Homo sapiens | |
67 | - |
ferricytochrome b5 | mutant P275L, 25°C, pH 7.0 | Homo sapiens | |
600 | - |
ferricytochrome b5 | wild-type, 25°C, pH 7.0 | Homo sapiens |