Genomes and Genes
Summary: A flavoprotein and iron sulfur-containing oxidoreductase that catalyzes the oxidation of NADH to NAD. In eukaryotes the enzyme can be found as a component of mitochondrial electron transport complex I. Under experimental conditions the enzyme can use CYTOCHROME C GROUP as the reducing cofactor. The enzyme was formerly listed as EC 184.108.40.206.
- Lin S, Gross U, Bohne W. Two internal type II NADH dehydrogenases of Toxoplasma gondii are both required for optimal tachyzoite growth. Mol Microbiol. 2011;82:209-21 pubmed publisher..Together, our studies indicate that although TgNDH2-I and TgNDH2-II are individually non-essential, the expression of both internal isoforms is required to maintain the mitochondrial physiology in T. gondii tachyzoites. ..
- Carilla Latorre S, Gallardo M, Annesley S, Calvo Garrido J, Grana O, Accari S, et al. MidA is a putative methyltransferase that is required for mitochondrial complex I function. J Cell Sci. 2010;123:1674-83 pubmed publisher..We found that these aspects of the phenotype are mediated by a chronic activation of AMPK, revealing a possible role of AMPK signaling in complex I cytopathology. ..
- Zhuang X, Cheng C. ND6 gene "lost" and found: evolution of mitochondrial gene rearrangement in Antarctic notothenioids. Mol Biol Evol. 2010;27:1391-403 pubmed publisher..Recently, the mitochondrial (mt) NADH dehydrogenase subunit 6 (ND6) gene and the adjacent transfer RNA(Glu) (tRNA(Glu)) were also reportedly lost...
- Maas M, Sellem C, Krause F, Dencher N, Sainsard Chanet A. Molecular gene therapy: overexpression of the alternative NADH dehydrogenase NDI1 restores overall physiology in a fungal model of respiratory complex I deficiency. J Mol Biol. 2010;399:31-40 pubmed publisher..fungal model Podospora anserina, that the overexpression of the native mitochondrial matrix-faced type II NADH dehydrogenase NDI1, paralogue of the human apoptosis inducing factor AIF1, can fully restore all physiological ..
- Tuppen H, Hogan V, He L, Blakely E, Worgan L, Al Dosary M, et al. The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families. Brain. 2010;133:2952-63 pubmed publisher..Our results confirm that NDUFS2 is a mutational hotspot in Caucasian children with isolated complex I deficiency and recommend the routine diagnostic investigation of this gene in patients with Leigh or Leigh-like phenotypes. ..
- Makita R, Kokaze A, Ohtsu T, Ishikawa M, Matsunaga N, Karita K, et al. Longevity-associated NADH dehydrogenase subunit-2 237 Leu/Met polymorphism modulates the effects of daily alcohol drinking on yearly changes in serum total and LDL cholesterol in Japanese men. Acta Med Okayama. 2009;63:331-8 pubmed publisher..047). These results suggest that ND2-237Met has a beneficial impact on yearly changes in serum LDLC in non-daily drinkers but not in daily drinkers...
- Ono H, Nakamura H, Matsuzaki M. A NADH dehydrogenase ubiquinone flavoprotein is decreased in patients with dilated cardiomyopathy. Intern Med. 2010;49:2039-42 pubmed..All samples were analyzed by Western blotting. NADH dehydrogenase ubiquinone flavoprotein (NDUFV1) production in the myocardium decreased significantly with DCM, in ..
- Steimle S, Bajzath C, Dörner K, Schulte M, Bothe V, Friedrich T. Role of subunit NuoL for proton translocation by respiratory complex I. Biochemistry. 2011;50:3386-93 pubmed publisher..In addition, the same H+/e- stoichiometry is obtained with the variant lacking the C-terminal transmembraneous helix of NuoL, indicating its role in energy transmission. ..
- Efremov R, Sazanov L. Structure of the membrane domain of respiratory complex I. Nature. 2011;476:414-20 pubmed publisher..The structure indicates that proton translocation in complex I, uniquely, involves coordinated conformational changes in six symmetrical structural elements. ..
- Hébert Chatelain E, Jose C, Gutiérrez Cortés N, Dupuy J, Rocher C, Dachary Prigent J, et al. Preservation of NADH ubiquinone-oxidoreductase activity by Src kinase-mediated phosphorylation of NDUFB10. Biochim Biophys Acta. 2012;1817:718-25 pubmed publisher..These results suggest that the preservation of complex I function by mitochondrial Src kinase could be important in the development of the overall phenotype of cancer. ..
- Yamamoto H, Peng L, Fukao Y, Shikanai T. An Src homology 3 domain-like fold protein forms a ferredoxin binding site for the chloroplast NADH dehydrogenase-like complex in Arabidopsis. Plant Cell. 2011;23:1480-93 pubmed publisher..We propose that NDH functions as a PGR5-PGRL1 complex-independent Fd:plastoquinone oxidoreductase in chloroplasts and rename it the NADH dehydrogenase-like complex.
- Joshi A, Raju N, Rajini P. Microplate-based kinetic method for assay of mitochondrial NADH-- and succinate--cytochrome c reductase activities. Anal Biochem. 2011;415:209-11 pubmed publisher..The microplate format described here can be employed for rapid and simultaneous measurements of mitochondrial NADH-- and succinate--cytochrome c reductase activities in a large number of samples. ..
- Kokaze A, Ishikawa M, Matsunaga N, Karita K, Yoshida M, Ohtsu T, et al. NADH dehydrogenase subunit-2 237 Leu/Met polymorphism modulates the effects of coffee consumption on the risk of hypertension in middle-aged Japanese men. J Epidemiol. 2009;19:231-6 pubmedHabitual coffee consumption has been reported to lower blood pressure in the Japanese population. The NADH dehydrogenase subunit-2 237 leucine/methionine (ND2-237 Leu/Met) polymorphism is associated with longevity and modifies the effects ..
- Damm F, Bunke T, Thol F, Markus B, Wagner K, Göhring G, et al. Prognostic implications and molecular associations of NADH dehydrogenase subunit 4 (ND4) mutations in acute myeloid leukemia. Leukemia. 2012;26:289-95 pubmed publisherTo study the prevalence and prognostic importance of mutations in NADH dehydrogenase subunit 4 (ND4), a mitochondrial encoded transmembrane component of the electron transport chain respiratory Complex I, 452 AML patients were examined ..
- Peng L, Fukao Y, Fujiwara M, Shikanai T. Multistep assembly of chloroplast NADH dehydrogenase-like subcomplex A requires several nucleus-encoded proteins, including CRR41 and CRR42, in Arabidopsis. Plant Cell. 2012;24:202-14 pubmed publisherChloroplast NADH dehydrogenase-like complex (NDH) mediates photosystem I cyclic electron transport and chlororespiration in thylakoids...
- Nakamaru Ogiso E, Kao M, Chen H, Sinha S, Yagi T, Ohnishi T. The membrane subunit NuoL(ND5) is involved in the indirect proton pumping mechanism of Escherichia coli complex I. J Biol Chem. 2010;285:39070-8 pubmed publisher..The electron transfer activities were basically unaffected by EIPA in both control and mutants. Taken together, our data strongly indicate that the NuoL subunit is involved in the indirect coupling mechanism. ..
- Clason T, Ruiz T, Schagger H, Peng G, Zickermann V, Brandt U, et al. The structure of eukaryotic and prokaryotic complex I. J Struct Biol. 2010;169:81-8 pubmed publisher..The differences seen in the membrane arm may indicate differences in the regulatory mechanism of the enzyme in different species. ..
- Papa S, Rasmo D, Technikova Dobrova Z, Panelli D, Signorile A, Scacco S, et al. Respiratory chain complex I, a main regulatory target of the cAMP/PKA pathway is defective in different human diseases. FEBS Lett. 2012;586:568-77 pubmed publisher..Complex I dysfunction has, indeed, been found, to be associated with several human diseases. Knowledge of the pathogenetic mechanisms of these diseases can help to develop new therapeutic strategies. ..
- McKenzie M, Ryan M. Assembly factors of human mitochondrial complex I and their defects in disease. IUBMB Life. 2010;62:497-502 pubmed publisher..Defects in assembly factors which disrupt complex I assembly and contribute to human disease pathogenesis will also be discussed. ..
- Lecler R, Vigeolas H, Emonds Alt B, Cardol P, Remacle C. Characterization of an internal type-II NADH dehydrogenase from Chlamydomonas reinhardtii mitochondria. Curr Genet. 2012;58:205-16 pubmed publisher..Respiration and growth rates in heterotrophic conditions were significantly altered in the double mutants investigated, suggesting that Nda1 plays a role in the oxidation of matrix NADH in the absence of complex I...
- Zurita Rendón O, Shoubridge E. Early complex I assembly defects result in rapid turnover of the ND1 subunit. Hum Mol Genet. 2012;21:3815-24 pubmed publisher..These data suggest that the four early CI assembly factors have non-redundant functions in the assembly of a module that docks and stabilizes newly synthesized ND1, nucleating assembly of the holoenzyme. ..
- Haack T, Madignier F, Herzer M, Lamantea E, Danhauser K, Invernizzi F, et al. Mutation screening of 75 candidate genes in 152 complex I deficiency cases identifies pathogenic variants in 16 genes including NDUFB9. J Med Genet. 2012;49:83-9 pubmed publisher..The authors conclude that the high degree of genetic heterogeneity in complex I disorders warrants the implementation of unbiased genome-wide strategies for the complete molecular dissection of mitochondrial complex I deficiency. ..
- O Donnell A, Harvey L, McNeil B. The roles of the alternative NADH dehydrogenases during oxidative stress in cultures of the filamentous fungus Aspergillus niger. Fungal Biol. 2011;115:359-69 pubmed publisher..The findings of the present study help further our understanding of the central roles of these highly conserved enzymes within fungal metabolism under oxidative stress. ..
- Hoefs S, van Spronsen F, Lenssen E, Nijtmans L, Rodenburg R, Smeitink J, et al. NDUFA10 mutations cause complex I deficiency in a patient with Leigh disease. Eur J Hum Genet. 2011;19:270-4 pubmed publisher..These results indicate that NDUFA10 is a novel candidate gene to screen for disease-causing mutations in patients with complex I deficiency. ..
- Kokaze A, Ishikawa M, Matsunaga N, Karita K, Yoshida M, Ohtsu T, et al. Unexpected combined effects of NADH dehydrogenase subunit-2 237 Leu/Met polymorphism and green tea consumption on renal function in male Japanese health check-up examinees: a cross-sectional study. J Negat Results Biomed. 2013;12:17 pubmed publisherb>NADH dehydrogenase subunit-2 237 leucine/methionine (ND2-237 Leu/Met) polymorphism is associated with longevity in Japanese...
- Baptista C, V ncio R, Abdala S, Carranza J, Westenberger S, Silva M, et al. Differential transcription profiles in Trypanosoma cruzi associated with clinical forms of Chagas disease: Maxicircle NADH dehydrogenase subunit 7 gene truncation in asymptomatic patient isolates. Mol Biochem Parasitol. 2006;150:236-48 pubmed publisher..surface protease GP63, cyclophilin, some hypothetical proteins and the pre-edited maxicircle gene NADH dehydrogenase subunit 7 (ND7)...
- Mogi T, Matsushita K, Murase Y, Kawahara K, Miyoshi H, Ui H, et al. Identification of new inhibitors for alternative NADH dehydrogenase (NDH-II). FEMS Microbiol Lett. 2009;291:157-61 pubmed publisherIn bacterial membranes and plant, fungus and protist mitochondria, NADH dehydrogenase (NDH-II) serves as an alternative NADH : quinone reductase, a non-proton-pumping single-subunit enzyme bound to the membrane surface...
- Euro L, Belevich G, Verkhovsky M, Wikstrom M, Verkhovskaya M. Conserved lysine residues of the membrane subunit NuoM are involved in energy conversion by the proton-pumping NADH:ubiquinone oxidoreductase (Complex I). Biochim Biophys Acta. 2008;1777:1166-72 pubmed publisher..A tentative principle of proton translocation by Complex I is suggested based on electrostatic interactions of lysines in the membrane subunits. ..
- Bracco J, Capurro M, Lourenco de Oliveira R, Sallum M. Genetic variability of Aedes aegypti in the Americas using a mitochondrial gene: evidence of multiple introductions. Mem Inst Oswaldo Cruz. 2007;102:573-80 pubmed..Amplification and sequencing of a fragment of the mitochondrial NADH dehydrogenase subunit 4 gene identified 20 distinct haplotypes, of which 14 are exclusive to the Americas, four to African/..
- Imkamp F, Biegel E, Jayamani E, Buckel W, Muller V. Dissection of the caffeate respiratory chain in the acetogen Acetobacterium woodii: identification of an Rnf-type NADH dehydrogenase as a potential coupling site. J Bacteriol. 2007;189:8145-53 pubmed..could encode this activity revealed DNA fragments encoding subunits C and D of a membrane-bound Rnf-type NADH dehydrogenase that is a potential Na(+) pump...
- Kokaze A, Ishikawa M, Matsunaga N, Yoshida M, Satoh M, Teruya K, et al. NADH dehydrogenase subunit-2 237 Leu/Met polymorphism modifies the effects of alcohol consumption on risk for hypertension in middle-aged Japanese men. Hypertens Res. 2007;30:213-8 pubmedb>NADH dehydrogenase subunit-2 237 leucine/methionine (ND2-237 Leu/Met) polymorphism is associated with longevity in the Japanese population, and the ND2-237Met genotype may exert antiatherogenic effects...
- Bonnet C, Kaltimbacher V, Ellouze S, Augustin S, Benit P, Forster V, et al. Allotopic mRNA localization to the mitochondrial surface rescues respiratory chain defects in fibroblasts harboring mitochondrial DNA mutations affecting complex I or v subunits. Rejuvenation Res. 2007;10:127-44 pubmed..Therefore, mRNA sorting to the mitochondrial surface represents a powerful strategy that could ultimately be applied in human therapy and become available for an array of devastating disorders caused by mtDNA mutations. ..
- Kalnenieks U, Galinina N, Strazdina I, Kravale Z, Pickford J, Rutkis R, et al. NADH dehydrogenase deficiency results in low respiration rate and improved aerobic growth of Zymomonas mobilis. Microbiology. 2008;154:989-94 pubmed publisher..mobilis aerobic growth due to redirection of the NADH flux from respiration to ethanol synthesis, thus minimizing accumulation of toxic intermediates by contributing to the reduction of acetaldehyde to ethanol...
- Kruse S, Watt W, Marcinek D, Kapur R, Schenkman K, Palmiter R. Mice with mitochondrial complex I deficiency develop a fatal encephalomyopathy. Cell Metab. 2008;7:312-20 pubmed publisher..KO muscle has normal morphology but low NADH dehydrogenase activity and subsarcolemmal aggregates of mitochondria...
- Zhang J, Li X, Wang Y, Ji J, Yang F, Feng G, et al. Association study on the mitochondrial gene NDUFV2 and bipolar disorder in the Chinese Han population. J Neural Transm (Vienna). 2009;116:357-61 pubmed publisher..No other individual associations of SNPs with bipolar were detected. Our study indicated that the regions spanning from the promoter to the exon 2 may contain susceptible polymorphisms which predispose to bipolar disorder. ..
- Ishikawa K, Takenaga K, Akimoto M, Koshikawa N, Yamaguchi A, Imanishi H, et al. ROS-generating mitochondrial DNA mutations can regulate tumor cell metastasis. Science. 2008;320:661-4 pubmed publisher..These results indicate that mtDNA mutations can contribute to tumor progression by enhancing the metastatic potential of tumor cells...
- Krishnan K, Bender A, Taylor R, Turnbull D. A multiplex real-time PCR method to detect and quantify mitochondrial DNA deletions in individual cells. Anal Biochem. 2007;370:127-9 pubmed
- Vogel R, Dieteren C, van den Heuvel L, Willems P, Smeitink J, Koopman W, et al. Identification of mitochondrial complex I assembly intermediates by tracing tagged NDUFS3 demonstrates the entry point of mitochondrial subunits. J Biol Chem. 2007;282:7582-90 pubmed..Composition analysis showed that this event was accompanied by the incorporation of at least one mitochondrial DNA-encoded subunit, thereby revealing the first entry point of these subunits. ..
- Fernandez Moreira D, Ugalde C, Smeets R, Rodenburg R, Lopez Laso E, Ruiz Falco M, et al. X-linked NDUFA1 gene mutations associated with mitochondrial encephalomyopathy. Ann Neurol. 2007;61:73-83 pubmed..Mutations in the X-linked NDUFA1 gene result in complex I defect and encephalomyopathy. Assembly/stability analysis might give an explanation for the different clinical phenotypes and become useful for future diagnostic purposes. ..
- Xu C, Li P, Kennedy J, Green M, Hughes B, Cooke R, et al. Further support for association of the mitochondrial complex I subunit gene NDUFV2 with bipolar disorder. Bipolar Disord. 2008;10:105-10 pubmed publisher..While genetic variants of NDUFV2 may increase risk for BD, the role of its altered expression and the link to intracellular Ca(2+) abnormalities in BD remains equivocal. ..
- Roychoudhury P, Ghosh U, Bhattacharyya N, Chaudhuri K. Activation of mitochondrial promoter P(H)-binding protein in a radio-resistant Chinese hamster cell strain associated with Bcl-2. Biochem Biophys Res Commun. 2006;350:272-6 pubmed..These results showed that differential mitochondrial gene expression observed earlier in the radio-resistant M5 cells was due to enhanced interaction proteins with the promoters P(H) and mediated by the expression of Bcl2. ..
- Richardson J, Caudle W, Guillot T, Watson J, Nakamaru Ogiso E, Seo B, et al. Obligatory role for complex I inhibition in the dopaminergic neurotoxicity of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP). Toxicol Sci. 2007;95:196-204 pubmed..Furthermore, combined with reports of a complex I defect in Parkinson's disease (PD) patients, the present study affirms the utility of MPTP in understanding the molecular mechanisms underlying dopaminergic neurodegeneration in PD. ..
- Ben Shachar D, Karry R. Sp1 expression is disrupted in schizophrenia; a possible mechanism for the abnormal expression of mitochondrial complex I genes, NDUFV1 and NDUFV2. PLoS ONE. 2007;2:e817 pubmed
- DeCorby A, Gaskova D, Sayles L, Lemire B. Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease. Biochim Biophys Acta. 2007;1767:1157-63 pubmed..We have expressed the Saccharomyces cerevisiae NDI1 gene, which encodes a single subunit NADH dehydrogenase, in a strain of Caenorhabditis elegans with an impaired complex I...
- Washizuka S, Iwamoto K, Kakiuchi C, Bundo M, Kato T. Expression of mitochondrial complex I subunit gene NDUFV2 in the lymphoblastoid cells derived from patients with bipolar disorder and schizophrenia. Neurosci Res. 2009;63:199-204 pubmed publisher..02). Our study presented the further evidence of biological significance of NDUFV2 in BD and SZ. ..
- Dunning C, McKenzie M, Sugiana C, Lazarou M, Silke J, Connelly A, et al. Human CIA30 is involved in the early assembly of mitochondrial complex I and mutations in its gene cause disease. EMBO J. 2007;26:3227-37 pubmed..Our results indicate that CIA30 is a crucial component in the early assembly of complex I and mutations in its gene can cause mitochondrial disease. ..
- Díaz M, de Haro V, Muñoz R, Quiles M. Chlororespiration is involved in the adaptation of Brassica plants to heat and high light intensity. Plant Cell Environ. 2007;30:1578-85 pubmed..of thylakoid membranes using specific antibodies raised against the NDH-K subunit of the thylakoidal NADH dehydrogenase complex (NADH DH) and against plastid terminal oxidase (PTOX) revealed a higher amount of both proteins in B...
- Ochsenreiter T, Cipriano M, Hajduk S. Alternative mRNA editing in trypanosomes is extensive and may contribute to mitochondrial protein diversity. PLoS ONE. 2008;3:e1566 pubmed publisher..Using this data, we show that alternative editing of COXIII, ATPase subunit 6 (A6), and NADH dehydrogenase subunits 7, 8 and 9 (ND7, 8, 9) mRNAs can produce novel open reading frames (ORFs)...
- Ellouze S, Augustin S, Bouaita A, Bonnet C, Simonutti M, Forster V, et al. Optimized allotopic expression of the human mitochondrial ND4 prevents blindness in a rat model of mitochondrial dysfunction. Am J Hum Genet. 2008;83:373-87 pubmed publisher..Hence, these data provide the proof-of-principle that optimized allotopic expression can be an effective treatment for LHON, and they open the way to clinical studies on other devastating mitochondrial disorders. ..
- Marella M, Seo B, Nakamaru Ogiso E, Greenamyre J, Matsuno Yagi A, Yagi T. Protection by the NDI1 gene against neurodegeneration in a rotenone rat model of Parkinson's disease. PLoS ONE. 2008;3:e1433 pubmed publisher..Using the rotenone rat model, we investigated the protective effects of alternative NADH dehydrogenase (Ndi1) which we previously demonstrated to act as a replacement for complex I both in vitro and in vivo...