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Literature summary extracted from

  • Percy, M.J.; Crowley, L.J.; Boudreaux, J.; Barber, M.J.
    Expression of a novel P275L variant of NADH:cytochrome b5 reductase gives functional insight into the conserved motif important for pyridine nucleotide binding (2006), Arch. Biochem. Biophys., 447, 59-67.
    View publication on PubMed

Application

EC Number Application Comment Organism
1.6.2.2 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

EC Number Protein Variants Comment Organism
1.6.2.2 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]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
1.6.2.2 0.004
-
 ferricytochrome b5 wild-type, 25°C, pH 7.0 Homo sapiens
1.6.2.2 0.006
-
 ferricyanide mutant P275L, 25°C, pH 7.0 Homo sapiens
1.6.2.2 0.006
-
 NADH mutant P275L, cosubstrate ferricyanide, 25°C, pH 7.0 Homo sapiens
1.6.2.2 0.007
-
 ferricyanide wild-type, 25°C, pH 7.0 Homo sapiens
1.6.2.2 0.012
-
 ferricytochrome b5 mutant P275L, 25°C, pH 7.0 Homo sapiens
1.6.2.2 2.623
-
 NADH wild-type, cosubstrate ferricyanide, 25°C, pH 7.0 Homo sapiens

Organism

EC Number Organism UniProt Comment Textmining
1.6.2.2 Homo sapiens P00387 sequence of natural mutant gene from a patient with recessive congenital methemoglobinemia; patients with recessive congenital methemoglobinemia
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.6.2.2 2 ferricyanide + NADH
-
 Homo sapiens 2 ferrocyanide + NAD+ + H+
-
 ?
1.6.2.2 2 ferricytochrome b5 + NADH
-
 Homo sapiens 2 ferrocytochrome b5 + NAD+ + H+
-
 ?

Turnover Number [1/s]

EC Number Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
1.6.2.2 0.733
-
 NADH wild-type, cosubstrate ferricyanide, 25°C, pH 7.0 Homo sapiens
1.6.2.2 0.8
-
 NADH mutant P275L, cosubstrate ferricyanide, 25°C, pH 7.0 Homo sapiens
1.6.2.2 67
-
 ferricytochrome b5 mutant P275L, 25°C, pH 7.0 Homo sapiens
1.6.2.2 600
-
 ferricytochrome b5 wild-type, 25°C, pH 7.0 Homo sapiens