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Function
NADH-cytochrome b5 reductase (b5R) reduces methaemoglobin to functional haemoglobin[1].
Disease
b5R deficiency causes methaemoglobinemia[2].
Relevance
b5R and cytochrome b5 catalyze the reduction of the antibiotic drug sulfamethoxazole[3].
- ↑ Bando S, Takano T, Yubisui T, Shirabe K, Takeshita M, Nakagawa A. Structure of human erythrocyte NADH-cytochrome b5 reductase. Acta Crystallogr D Biol Crystallogr. 2004 Nov;60(Pt 11):1929-34. Epub 2004, Oct 20. PMID:15502298 doi:10.1107/S0907444904020645
- ↑ Aalfs CM, Salieb-Beugelaar GB, Wanders RJ, Mannens MM, Wijburg FA. A case of methemoglobinemia type II due to NADH-cytochrome b5 reductase deficiency: determination of the molecular basis. Hum Mutat. 2000;16(1):18-22. doi:, 10.1002/1098-1004(200007)16:1<18::AID-HUMU4>3.0.CO;2-N. PMID:10874300 doi:<18::AID-HUMU4>3.0.CO;2-N https://dx.doi.org/10.1002/1098-1004(200007)16:1<18::AID-HUMU4>3.0.CO;2-N
- ↑ Sacco JC, Trepanier LA. Cytochrome b5 and NADH cytochrome b5 reductase: genotype-phenotype correlations for hydroxylamine reduction. Pharmacogenet Genomics. 2010 Jan;20(1):26-37. doi: 10.1097/FPC.0b013e3283343296. PMID:19997042 doi:https://dx.doi.org/10.1097/FPC.0b013e3283343296
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3D structures of NADH-cytochrome b5 reductase
Updated on 15-January-2026
1umk, 7thg, 7two, 7w3o - hb5R3 + FAD - human
7tnv, 7tsw - hb5R3 (mutant) + FAD
1ndh, 3w2e, 3w2f, 3w2g, 3w2h, 3w2i, 3w5h, 5gv7, 5gv8, 9v69 - pb5R 3 + FAD + NAD - pig
9v6a - pb5R 3 (mutant) + FAD + NAD
9v6b, 9v6c - pb5R 3 + FAD - neutron
1i7p - rb5R soluble domain + FAD - rat
1qx4 - rb5R soluble domain (mutant) + FAD
1ib0 - rb5R soluble domain + FAD + NAD
7rom - b5R (mutant) + FAD - yeast
2eix - b5R + FAD - Physarum polycephalum
References
