Literature summary for 1.6.2.2 extracted from

Percy, M.J.; Crowley, L.J.; Roper, D.; Vulliamy, T.J.; Layton, D.M.; Barber, M.J.
Identification and characterization of the novel FAD-binding lobe G75S mutation in cytochrome b(5) reductase: an aid to determine recessive congenital methemoglobinemia status in an infant (2006), Blood Cells Mol. Dis., 36, 81-90.

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Protein Variants

Protein Variants	Comment	Organism
G75S	natural mutation isolated in patient with recessive congenital methemoglobinaemia. Mutant retains stoichiometric levels of FAD, but shows decreased catalytic efficiency and reduced protein stability	Homo sapiens
G75S/V252M	natural mutation isolated in patient with recessive congenital methemoglobinaemia. Mutant retains stoichiometric levels of FAD, but shows decreased catalytic efficiency and reduced protein stability	Homo sapiens
V252M	natural mutation isolated in patient with recessive congenital methemoglobinaemia. Mutant retains stoichiometric levels of FAD, but shows decreased catalytic efficiency and reduced protein stability	Homo sapiens

KM Value [mM]

KM Value [mM]	KM Value Maximum [mM]	Substrate	Comment	Organism	Structure
0.006	-	NADH	wild-type	Homo sapiens
0.006	-	ferricyanide	wild-type	Homo sapiens
0.007	-	ferricyanide	mutant G75S	Homo sapiens
0.007	-	ferricyanide	mutant V252M	Homo sapiens
0.008	-	ferricyanide	mutant G75S/V252M	Homo sapiens
0.009	-	NADH	mutant G75S	Homo sapiens
0.013	-	ferricytochrome b5	wild-type	Homo sapiens
0.013	-	ferricytochrome b5	mutant V252M	Homo sapiens
0.014	-	ferricytochrome b5	mutant G75S	Homo sapiens
0.014	-	ferricytochrome b5	mutant G75S/V252M	Homo sapiens
0.017	-	NADH	mutant G75S/V252M	Homo sapiens
0.052	-	NADH	mutant V252M	Homo sapiens

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	-	-	-

Substrates and Products (Substrate)

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 [1/s]

Turnover Number Minimum [1/s]	Turnover Number Maximum [1/s]	Substrate	Comment	Organism	Structure
60	-	ferricytochrome b5	mutant G75S	Homo sapiens
85	-	ferricytochrome b5	mutant G75S/V252M	Homo sapiens
133	-	ferricyanide	mutant G75S	Homo sapiens
187	-	ferricyanide	mutant G75S/V252M	Homo sapiens
285	-	ferricytochrome b5	mutant V252M	Homo sapiens
360	-	ferricytochrome b5	wild-type	Homo sapiens
633	-	ferricyanide	mutant V252M	Homo sapiens
800	-	ferricyanide	wild-type	Homo sapiens

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Release 2023.1 (January 2023)