NADH dehydrogenase; respiratory chain complex-I; NADH CoQ reductase;NADH-ubiquinone oxidoreductase

Function: - multienzyme complex 1 of mitochondrial electron transport NADH + ubiquinone NAD⁺ + ubiquinol NADH + acceptor NAD⁺ + reduced acceptor Structure: - an estimated 45 subunits - subunits encoded by mitochondrial (7-13) & nuclear (> 20) genes Pathology: - defects in subunits associated with NADH dehydrogenase deficiency

Related

1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) electron transport NADH dehydrogenase deficiency (complex 1 mitochondrial respiratory chain deficiency) NADH:ubiquinone reductase in specimen

General

cytochrome reductase dehydrogenase electron transport enzyme multienzyme complex quinone reductase

Properties

SIZE: MW = 850 kD COMPARTMENT: inner mitochondrial membrane MOTIF: heme-binding site

Database Correlations

OMIM 602137 ENZYME 1.6.5.3

Components

NADH dehydrogenase 13 kD subunit, mitochondrial-encoded (complex 1-13KD, NADH ubiquinone oxidoreductase subunit FeS protein 6, NDUFS6) NADH dehydrogenase 15 kD subunit (NADH dehydrogenase [ubiquinone] iron-sulfur protein 5, NADH ubiquinone oxidoreductase subunit FeS protein 5, complex 1-15 kD, C1-15 kD, NDUFS5) NADH dehydrogenase 18 kD subunit, mitochondrial-encoded (complex 1-18KD, complex 1-AQDQ, NADH ubiquinone oxidoreductase subunit FeS protein 4, NDUFS4) NADH dehydrogenase 19 kD subunit (NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8, complex 1-19KD, Complex 1-PGIV, NDUFA8) NADH dehydrogenase 20 kD subunit, mitochondrial-encoded (complex 1-20KD, NADH ubiquinone oxidoreductase subunit FeS protein 7, NDUFS7, PSST subunit) NADH dehydrogenase 30 kD subunit, mitochondrial-encoded (complex 1-30KD, NADH ubiquinone oxidoreductase subunit FeS protein 3, NDUFS3, PSST subunit) NADH dehydrogenase 39 kD subunit, mitochondrial-encoded (NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, complex 1-39KD, NDUFA9, NDUFS2L) NADH dehydrogenase 49 kD subunit, mitochondrial-encoded (complex 1-49KD, NADH ubiquinone oxidoreductase subunit FeS protein 2, NDUFS2) NADH dehydrogenase 51 kD subunit, mitochondrial-encoded (complex 1-51KD, NADH ubiquinone oxidoreductase flavoprotein 1, NDUFV1, UQOR1) NADH dehydrogenase 9 kD subunit, mitochondrial-encoded (complex 1-9kD, NADH ubiquinone oxidoreductase flavoprotein 3, NDUFV3) NADH dehydrogenase AGGG subunit, mitochondrial-encoded (NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, complex 1-AGGG, NDUFB2) NADH dehydrogenase ASHI subunit, mitochondrial-encoded (NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, complex 1-ASHI, NDUFB8) NADH dehydrogenase B12 (NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3, complex 1-B12, CI-B12, NDUFB3) NADH dehydrogenase B13 (Complex 1-B13, NADH ubiquinone oxidoreductase alpha subcomplex 5, NDUFA5) NADH dehydrogenase B14 (NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6, complex 1-B14, NDUFA6) NADH dehydrogenase B14.5A (NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7, complex 1-B14.5, C1- B14.5a NDUFA7) NADH dehydrogenase B14.5B (NADH dehydrogenase [ubiquinone] 1 subunit C2, complex 1-B14.5B, NDUFC2) NADH dehydrogenase B15; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4; complex 1-B15; C1-B15 (NDUFB4) NADH dehydrogenase B16.6 (NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13, complex 1-B16.6, C1-B16.6, gene associated with retinoic-interferon-induced mortality 19 protein, GRIM-19, cell death-regulatory protein, CGI-39, CDA016, NDUFA13) NADH dehydrogenase B17 (NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6,complex 1-B17, NADH ubiquinone oxidoreductase beta subcomplex 6, NDUFB6) NADH dehydrogenase B17.2 (NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12, complex 1-B17.2, 13 kD differentiation-associated antigen, DAP13, NDUFA12) NADH dehydrogenase B22 (complex 1-B22, NADH ubiquinone oxidoreductase beta subcomplex 9, LYR motif-containing protein 3, NDUFB9) NADH dehydrogenase B8 (complex 1-B8, NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2, CI-B8, NDUFA2) NADH dehydrogenase B9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3; complex 1-B9; CI-B9 (NDUFA3) NADH dehydrogenase KFYI subunit; mitochondrial-encoded NADH dehydrogenase [ubiquinone] 1 subunit C1; complex 1-KFYI (NDUFC1) NADH dehydrogenase MLRQ subunit (NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4, complex 1-MLRQ, C1-MLRQ, NDUFA4) NADH dehydrogenase MNLL subunit (NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1, complex 1-MNLL, C1-MNLL, NDUFB1) NADH dehydrogenase MWFE subunit (NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1, complex 1-MWFE, NDUFA1) NADH dehydrogenase p24 (flavoprotein 2, NADH ubiquinone oxidoreductase p24, NDUFV2) NADH dehydrogenase p25 (NADH ubiquinone oxidoreductase p25) NADH dehydrogenase p30 (NADH ubiquinone oxidoreductase p30) NADH dehydrogenase p49 (NADH ubiquinone oxidoreductase p49) NADH dehydrogenase p75 (NADH ubiquinone oxidoreductase p75, NDUFS1, complex I-75kD, CI-75kD) NADH dehydrogenase PDSW subunit (NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10, complex 1-PDSW, C1-PDSW, NDUFB10) NADH dehydrogenase SGDH subunit, mitochondrial-encoded; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial; NADH-ubiquinone oxidoreductase SGDH subunit; complex 1-SGDH; C1-SGDH (NDUFB5) NADH dehydrogenase subunit-1 (NADH ubiquinone oxidoreductase chain-1, MT-ND1, MTND1, NADH1, ND1) NADH dehydrogenase subunit-2 (MT-ND2) NADH dehydrogenase subunit-3 (MT-ND3) NADH dehydrogenase subunit-4 NADH dehydrogenase subunit-4L (MT-ND4L) NADH dehydrogenase subunit-5 (MT-ND5) NADH dehydrogenase subunit-6 (MT-ND6) NADH dehydrogenase [ubiquinone] 1 subunit C2, isoform 2; NDUFC2-KCTD14 readthrough transcript protein (NDUFC2-KCTD14) protein subunit