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NADH-ubiquinone oxidoreductase chain 1 (EC 1.6.5.3) (NADH dehydrogenase subunit 1)

 NU1M_MURSU              Reviewed;         318 AA.
Q8M867;
12-APR-2005, integrated into UniProtKB/Swiss-Prot.
01-OCT-2002, sequence version 1.
16-JAN-2019, entry version 55.
RecName: Full=NADH-ubiquinone oxidoreductase chain 1;
EC=7.1.1.2;
AltName: Full=NADH dehydrogenase subunit 1;
Name=MT-ND1; Synonyms=MTND1, NADH1, ND1;
Murina suilla (Brown tube-nosed bat) (Lesser tube-nosed bat).
Mitochondrion.
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
Mammalia; Eutheria; Laurasiatheria; Chiroptera; Microchiroptera;
Vespertilionidae; Murina.
NCBI_TaxID=59489;
[1]
NUCLEOTIDE SEQUENCE [GENOMIC DNA].
PubMed=12187382; DOI=10.1007/s00239-002-2326-0;
Kawai K., Nikaido M., Harada M., Matsumura S., Lin L.K., Wu Y.,
Hasegawa M., Okada N.;
"Intra- and interfamily relationships of Vespertilionidae inferred by
various molecular markers including SINE insertion data.";
J. Mol. Evol. 55:284-301(2002).
-!- FUNCTION: Core subunit of the mitochondrial membrane respiratory
chain NADH dehydrogenase (Complex I) that is believed to belong to
the minimal assembly required for catalysis. Complex I functions
in the transfer of electrons from NADH to the respiratory chain.
The immediate electron acceptor for the enzyme is believed to be
ubiquinone (By similarity). {ECO:0000250}.
-!- CATALYTIC ACTIVITY:
Reaction=a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4
H(+)(out) + NAD(+); Xref=Rhea:RHEA:29091, Rhea:RHEA-COMP:9565,
Rhea:RHEA-COMP:9566, ChEBI:CHEBI:15378, ChEBI:CHEBI:16389,
ChEBI:CHEBI:17976, ChEBI:CHEBI:57540, ChEBI:CHEBI:57945;
EC=7.1.1.2;
-!- SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000250};
Multi-pass membrane protein {ECO:0000250}.
-!- SIMILARITY: Belongs to the complex I subunit 1 family.
{ECO:0000305}.
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Distributed under the Creative Commons Attribution (CC BY 4.0) License
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EMBL; AB079835; BAB92060.1; -; Genomic_DNA.
HOVERGEN; HBG016682; -.
GO; GO:0016021; C:integral component of membrane; IEA:UniProtKB-KW.
GO; GO:0005743; C:mitochondrial inner membrane; IEA:UniProtKB-SubCell.
GO; GO:0070469; C:respirasome; IEA:UniProtKB-KW.
GO; GO:0055114; P:oxidation-reduction process; IEA:UniProtKB-KW.
HAMAP; MF_01350; NDH1_NuoH; 1.
InterPro; IPR001694; NADH_UbQ_OxRdtase_su1/FPO.
InterPro; IPR018086; NADH_UbQ_OxRdtase_su1_CS.
PANTHER; PTHR11432; PTHR11432; 1.
Pfam; PF00146; NADHdh; 1.
PROSITE; PS00667; COMPLEX1_ND1_1; 1.
PROSITE; PS00668; COMPLEX1_ND1_2; 1.
3: Inferred from homology;
Electron transport; Membrane; Mitochondrion;
Mitochondrion inner membrane; NAD; Respiratory chain; Translocase;
Transmembrane; Transmembrane helix; Transport; Ubiquinone.
CHAIN 1 318 NADH-ubiquinone oxidoreductase chain 1.
/FTId=PRO_0000117432.
TRANSMEM 3 23 Helical. {ECO:0000255}.
TRANSMEM 69 89 Helical. {ECO:0000255}.
TRANSMEM 102 122 Helical. {ECO:0000255}.
TRANSMEM 144 164 Helical. {ECO:0000255}.
TRANSMEM 171 191 Helical. {ECO:0000255}.
TRANSMEM 222 242 Helical. {ECO:0000255}.
TRANSMEM 253 273 Helical. {ECO:0000255}.
TRANSMEM 294 314 Helical. {ECO:0000255}.
SEQUENCE 318 AA; 35860 MW; FE93F9D119543389 CRC64;
MYLINLLAMI VPVLLAVAFL TLLERKVLGY MQLRKGPNIV GPYGLLQPIA DAVKLFTKEP
LQPLTSSPML FIIAPTLALT LALTMWTPLP MPYPLMNMNL SMLFILALSS LAVYTIMWSG
WASNSKYALI GALRAVAQTI SYEVTLAIII LSLLLMNGSF TLLSMTTTQE YIWLLIPSWP
LAMMWFISTL AETNRAPFDL TEGESELVSG FNVEYAGGPF ALFFLAEYAN IIMMNALTII
LFLGAYHNSM VPELYTINFT IKTLLFTAFF LWIRASYPRF RYDQLMHLLW KNFLPLTLVM
CMWHVALPIM LAGIPPQT


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Pathways :
WP1566: Citrate cycle (TCA cycle)
WP1614: 1- and 2-Methylnaphthalene degradation
WP1626: Benzoate degradation via CoA ligation
WP1665: Limonene and pinene degradation
WP1567: Glycolysis and Gluconeogenesis
WP1627: Benzoate degradation via hydroxylation
WP1633: Bisphenol A degradation
WP1634: Butanoate metabolism
WP1647: Fatty acid biosynthesis
WP1654: gamma-Hexachlorocyclohexane degradation
WP1655: Geraniol degradation
WP1671: Methane metabolism
WP1673: Naphthalene and anthracene degradation
WP1680: Oxidative phosphorylation
WP1695: Pyruvate metabolism
WP1711: Trinitrotoluene degradation
WP1717: Valine, leucine and isoleucine degradation
WP1002: Electron Transport Chain
WP111: Electron Transport Chain
WP1119: Electron Transport Chain
WP1339: Electron Transport Chain
WP1493: Carbon assimilation C4 pathway
WP1502: Mitochondrial biogenesis
WP1581: Histidine metabolism
WP1611: 1,1,1-Trichloro-2,2-bis(4-chlorophenyl)ethane (DDT

Related Genes :
[NDUFV1 UQOR1] NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial (EC 1.6.99.3) (EC 7.1.1.2) (Complex I-51kD) (CI-51kD) (NADH dehydrogenase flavoprotein 1) (NADH-ubiquinone oxidoreductase 51 kDa subunit)
[MT-ND6 MTND6 NADH6 ND6] NADH-ubiquinone oxidoreductase chain 6 (EC 7.1.1.2) (NADH dehydrogenase subunit 6)
[MT-ND5 MTND5 NADH5 ND5] NADH-ubiquinone oxidoreductase chain 5 (EC 7.1.1.2) (NADH dehydrogenase subunit 5)
[NDUFS7] NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial (EC 1.6.99.3) (EC 7.1.1.2) (Complex I-20kD) (CI-20kD) (NADH-ubiquinone oxidoreductase 20 kDa subunit) (PSST subunit)
[MT-ND1 MTND1 NADH1 ND1] NADH-ubiquinone oxidoreductase chain 1 (EC 7.1.1.2) (NADH dehydrogenase subunit 1)
[NDUFV1 UQOR1] NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial (EC 1.6.99.3) (EC 7.1.1.2) (Complex I-51kD) (CI-51kD) (NADH dehydrogenase flavoprotein 1) (NADH-ubiquinone oxidoreductase 51 kDa subunit)
[MT-ND3 MTND3 NADH3 ND3] NADH-ubiquinone oxidoreductase chain 3 (EC 7.1.1.2) (NADH dehydrogenase subunit 3)
[NDUFS8] NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial (EC 1.6.99.3) (EC 7.1.1.2) (Complex I-23kD) (CI-23kD) (NADH-ubiquinone oxidoreductase 23 kDa subunit) (TYKY subunit)
[NDUFS2] NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial (EC 1.6.99.3) (EC 7.1.1.2) (Complex I-49kD) (CI-49kD) (NADH-ubiquinone oxidoreductase 49 kDa subunit)
[nuo78 B17C10.90 NCU01765] NADH-ubiquinone oxidoreductase 78 kDa subunit, mitochondrial (EC 1.6.99.3) (EC 7.1.1.2) (Complex I-78kD) (CI-78kD)
[NDC1 At5g08740 T2K12.12 T2K12_90] Alternative NAD(P)H-ubiquinone oxidoreductase C1, chloroplastic/mitochondrial (EC 1.6.5.9) (Alternative NADH dehydrogenase NDC1) (Demethylphylloquinone reductase NDC1) (EC 1.6.5.12) (NADH:ubiquinone reductase (non-electrogenic) NDC1)
[MT-ND4 MTND4 NADH4 ND4] NADH-ubiquinone oxidoreductase chain 4 (EC 7.1.1.2) (NADH dehydrogenase subunit 4)
[MT-ND2 MTND2 NADH2 ND2] NADH-ubiquinone oxidoreductase chain 2 (EC 7.1.1.2) (NADH dehydrogenase subunit 2)
[NDUFS8] NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial (EC 1.6.99.3) (EC 7.1.1.2) (Complex I-23kD) (CI-23kD) (NADH-ubiquinone oxidoreductase 23 kDa subunit) (TYKY subunit)
[NDUFV2] NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial (EC 1.6.99.3) (EC 7.1.1.2) (NADH dehydrogenase subunit II) (NADH-ubiquinone oxidoreductase 24 kDa subunit)
[nuo-51 B10H18.210 NCU04044] NADH-ubiquinone oxidoreductase 51 kDa subunit, mitochondrial (EC 1.6.99.3) (EC 7.1.1.2) (Complex I-51kD) (CI-51kD)
[NDUFS1] NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial (EC 1.6.99.3) (EC 7.1.1.2) (Complex I-75kD) (CI-75kD)
[NDB1 At4g28220 F26K10.100] External alternative NAD(P)H-ubiquinone oxidoreductase B1, mitochondrial (EC 1.6.5.9) (External alternative NADH dehydrogenase NDB1) (NADH:ubiquinone reductase (non-electrogenic) NDB1)
[nuoC nuoCD nuoD b2286 JW5375] NADH-quinone oxidoreductase subunit C/D (EC 7.1.1.-) (NADH dehydrogenase I subunit C/D) (NDH-1 subunit C/D) (NUO3/NUO4)
[EMB1467 At5g37510 MPA22.5] NADH dehydrogenase [ubiquinone] iron-sulfur protein 1, mitochondrial (EC 1.6.99.3) (EC 7.1.1.2) (Protein EMBRYO DEFECTIVE 1467)
[nuo-49 NCU02534] NADH-ubiquinone oxidoreductase 49 kDa subunit, mitochondrial (EC 1.6.99.3) (EC 7.1.1.2) (Complex I-49kD) (CI-49kD)
[NDA1 NDI1 At1g07180 F10K1.11] Internal alternative NAD(P)H-ubiquinone oxidoreductase A1, mitochondrial (EC 1.6.5.9) (Internal alternative NADH dehydrogenase NDA1) (Internal non-phosphorylating NAD(P)H dehydrogenase 1) (AtNDI1) (NADH:ubiquinone reductase (non-electrogenic) NDA1)
[NDUFS7] NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial (EC 1.6.99.3) (EC 7.1.1.2) (Complex I-20kD) (CI-20kD) (NADH-ubiquinone oxidoreductase 20 kDa subunit) (PSST subunit)
[nuoG b2283 JW2278] NADH-quinone oxidoreductase subunit G (EC 7.1.1.-) (NADH dehydrogenase I subunit G) (NDH-1 subunit G) (NUO7)
[nqo3 TTHA0090] NADH-quinone oxidoreductase subunit 3 (EC 7.1.1.-) (NADH dehydrogenase I chain 3) (NDH-1 subunit 3)
[Hsd3b1 Hsd3b] 3 beta-hydroxysteroid dehydrogenase/Delta 5-->4-isomerase type 1 (3 beta-hydroxysteroid dehydrogenase/Delta 5-->4-isomerase type I) (3-beta-HSD I) (3-beta-hydroxy-5-ene steroid dehydrogenase) (3-beta-hydroxy-Delta(5)-steroid dehydrogenase) (EC 1.1.1.145) (3-beta-hydroxysteroid 3-dehydrogenase) (EC 1.1.1.270) (Delta-5-3-ketosteroid isomerase) (Dihydrotestosterone oxidoreductase) (EC 1.1.1.210) (Steroid Delta-isomerase) (EC 5.3.3.1)
[nuoK b2279 JW2274] NADH-quinone oxidoreductase subunit K (EC 7.1.1.-) (NADH dehydrogenase I subunit K) (NDH-1 subunit K) (NUO11)
[NDB2 At4g05020 T32N4.4] External alternative NAD(P)H-ubiquinone oxidoreductase B2, mitochondrial (EC 1.6.5.9) (External alternative NADH dehydrogenase NDB2) (NADH:ubiquinone reductase (non-electrogenic) NDB2)
[NDUFA13 GRIM19] NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 (Cell death regulatory protein GRIM-19) (Complex I-B16.6) (CI-B16.6) (Gene associated with retinoic-interferon-induced mortality 19 protein) (GRIM-19) (NADH-ubiquinone oxidoreductase B16.6 subunit)
[nuoN b2276 JW2271] NADH-quinone oxidoreductase subunit N (EC 7.1.1.-) (NADH dehydrogenase I subunit N) (NDH-1 subunit N) (NUO14)

Bibliography :
[30326946] A cryptic species of Onchocerca (Nematoda: Onchocercidae) in blackflies (Simulium spp.) from southern California, USA.
[29948731] Cystic Leucoencephalopathy in NDUFV1 Mutation.
[29523164] Mitochondrial genome data confirm that yaks can serve as the intermediate host of Echinococcus canadensis (G10) on the Tibetan Plateau.
[29518970] Unravelling the Effects of the Mutation m.3571insC/MT-ND1 on Respiratory Complexes Structural Organization.
[29348607] Evaluation of mitochondrial bioenergetics, dynamics, endoplasmic reticulum-mitochondria crosstalk, and reactive oxygen species in fibroblasts from patients with complex I deficiency.
[29333085] Next Generation Sequencing expression profiling of mitochondrial subunits in men with Klinefelter syndrome.
[29096719] Subunit composition of respiratory chain complex 1 and its responses to oxygen in mitochondria from human donor livers.
[29095828] Molecular discrimination of Opisthorchis-like eggs from residents in a rural community of central Thailand.
[28696810] Mitochondrial DNA variations are associated with recurrent pregnancy loss.
[28647203] Gene Therapy for Leber Hereditary Optic Neuropathy: Low- and Medium-Dose Visual Results.
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