| Name | ND4 |
|---|---|
| Synonyms | MT ND4; MTND 4; MTND4; NADH dehydrogenase 4; NADH dehydrogenase subunit 4; NADH ubiquinone oxidoreductase chain 4; ND4; mitochondrially encoded NADH dehydrogenase 4… |
| Name | rotenone |
|---|---|
| CAS |
| PubMed | Abstract | RScore(About this table) | |
|---|---|---|---|
| 18291703 | Dlaskova A, Hlavata L, Jezek P: Oxidative stress caused by blocking of mitochondrial complex I H (+) pumping as a link in aging/disease vicious cycle. Int J Biochem Cell Biol. 2008;40(9):1792-805. Epub 2008 Jan 19. H (+) pumping may be attenuated by high protonmotive force or inhibited by oxidative stress-related mutations of ND5 (ND2, ND4) subunit. Rotenone caused a 5-fold J (m) increase (AC (50) 2 microM), which was attenuated by uncoupling, membrane potential (DeltaPsi (m)), and DeltapH-collapse, since addition of FCCP (IC (50) 55 nM), valinomycin, and nigericin prevented this increase. |
1(0,0,0,1) | Details |
| 15342361 | Beretta S, Mattavelli L, Sala G, Tremolizzo L, Schapira AH, Martinuzzi A, Carelli V, Ferrarese C: Leber hereditary optic neuropathy mtDNA mutations disrupt transport in cybrid cell lines. Brain. 2004 Oct;127(Pt 10):2183-92. Epub 2004 Sep 1. Three pathogenic mutations (positions 11778/ND4, 3460/ND1 and 14484/ND6) account for the majority of LHON cases and they affect genes that encode for different subunits of mitochondrial complex I. |
1(0,0,0,1) | Details |
| 12379308 | Carelli V, Vergani L, Bernazzi B, Zampieron C, Bucchi L, Valentino M, Rengo C, Torroni A, Martinuzzi A: Respiratory function in cybrid cell lines carrying European mtDNA haplogroups: implications for Leber's hereditary optic neuropathy. Biochim Biophys Acta. 2002 Oct 9;1588(1):7-14. Different possible explanations for the previously established association between haplogroup J and LHON 11778/ND4 and 14484/ND6 pathogenic mutations are discussed, including the unconventional proposal that mtDNA haplogroup J may exert a protective rather than detrimental effect. |
1(0,0,0,1) | Details |
| 1332758 | Finel M, Skehel JM, Albracht SP, Fearnley IM, Walker JE: Resolution of NADH:ubiquinone oxidoreductase from bovine heart mitochondria into two subcomplexes, one of which contains the redox centers of the enzyme. Biochemistry. 1992 Nov 24;31(46):11425-34. The line shapes of the EPR spectra of the Fe-S clusters are slightly broadened relative to spectra measured on complex I purified by conventional means, and the quinone reductase activity is insensitive to rotenone. The sequences of many of them contain hydrophobic segments that could be membrane spanning, including at least two mitochondrial gene products, ND4 and ND5. |
1(0,0,0,1) | Details |
| 11358527 | Fang J, Wang Y, Beattie DS: Isolation and characterization of complex I, rotenone-sensitive oxidoreductase, from the procyclic forms of Trypanosoma brucei. Eur J Biochem. 2001 May;268(10):3075-82. Four polypeptides of the partially purified enzyme were identified as the homologous subunits of complex I (51 kDa, PSST, TYKY, and ND4) by immunoblotting with antibodies raised against subunits of Paracoccus denitrificans and against synthetic peptides predicted from putative complex I subunit genes encoded by mitochondrial and nuclear T. brucei DNA. |
1(0,0,0,1) | Details |
| 8662757 | Hofhaus G, Johns DR, Hurko O, Attardi G, Chomyn A: Respiration and growth defects in transmitochondrial cell lines carrying the 11778 mutation associated with Leber's hereditary optic neuropathy. J Biol Chem. 1996 May 31;271(22):13155-61. On the contrary, no decrease in rotenone-sensitive NADH dehydrogenase activity, using a water-soluble analogue as electron acceptor, was detected in disrupted mitochondrial membranes. |
0(0,0,0,0) | Details |
| 11479321 | Bai Y, Hajek P, Chomyn A, Chan E, Seo BB, Matsuno-Yagi A, Yagi T, Attardi G: Lack of complex I activity in human cells carrying a mutation in MtDNA-encoded ND4 subunit is corrected by the Saccharomyces cerevisiae -quinone oxidoreductase (NDI1) gene. J Biol Chem. 2001 Oct 19;276(42):38808-13. Epub 2001 Jul 30. The gene for the single subunit, rotenone-insensitive, and -sensitive internal -quinone oxidoreductase of Saccharomyces cerevisiae (NDI1) can completely restore the NADH dehydrogenase activity in mutant human cells that lack the essential mitochondrial DNA (mtDNA)-encoded subunit ND4. |
8(0,0,1,3) | Details |
| 9191778 | Carelli V, Ghelli A, Ratta M, Bacchilega E, Sangiorgi S, Mancini R, Leuzzi V, Cortelli P, Montagna P, Lugaresi E, Degli Esposti M: Leber's hereditary optic neuropathy: biochemical effect of 11778/ND4 and 3460/ND1 mutations and correlation with the mitochondrial genotype. Neurology. 1997 Jun;48(6):1623-32. In platelets homoplasmic for mutant mtDNA, both 11778/ND4 and 3460/ND1 mutations induced resistance to rotenone and the 3460/ND1 mutation also provoked a marked decrease in the specific activity of complex I. |
8(0,0,1,3) | Details |
| 9266534 | Ghelli A, Degli Esposti M, Carelli V, Lenaz G: Changes in mitochondrial complex I activity and binding site in Leber's hereditary optic neuropathy (LHON). Mol Aspects Med. 1997;18 Suppl:S263-7. Both 11778/ND4 and 3460/ND1 mutations induced rotenone resistance and 11778/ND4 showed an increased K (m) for -2 with respect to the control group. |
7(0,0,1,2) | Details |
| 18235013 | Ghelli A, Porcelli AM, Zanna C, Martinuzzi A, Carelli V, Rugolo M: Protection against oxidant-induced apoptosis by exogenous in Leber hereditary optic neuropathy cybrids. Invest Ophthalmol Vis Sci. 2008 Feb;49(2):671-6. METHODS: Cybrids bearing one of the three most common LHON pathogenic mutations (11778/ND4, 3460/ND1, 14484/ND6) were incubated with two compounds known to induce oxidative injury, tert-butyl hydroperoxide (t-BH) and rotenone. |
6(0,0,1,1) | Details |
| 1959619 | Majander A, Huoponen K, Savontaus ML, Nikoskelainen E, Wikstrom M: Electron transfer properties of NADH:ubiquinone reductase in the ND1/3460 and the ND4/11778 mutations of the Leber hereditary optic neuroretinopathy (LHON). FEBS Lett. 1991 Nov 4;292(1-2):289-92. The ND1/3460 mutation exhibits 80% reduction in rotenone-sensitive and -dependent electron transfer activity, whereas the proximal NADH dehydrogenase activity of the Complex is unaffected. |
3(0,0,0,3) | Details |
| 12079358 | Cardol P, Matagne RF, Remacle C: Impact of mutations affecting ND mitochondria-encoded subunits on the activity and assembly of complex I in Chlamydomonas. J Mol Biol. 2002 Jun 21;319(5):1211-21. The mitochondrial rotenone-sensitive NADH:ubiquinone oxidoreductase (complex I) comprises more than 35 subunits, the majority of which are encoded by the nucleus. In Chlamydomonas reinhardtii, only five components (ND1, ND2, ND4, ND5 and ND6) are coded for by the mitochondrial genome. |
2(0,0,0,2) | Details |
| 7823960 | Hofhaus G, Attardi G: Efficient selection and characterization of mutants of a human cell line which are defective in mitochondrial DNA-encoded subunits of respiratory NADH dehydrogenase. Mol Cell Biol. 1995 Feb;15(2):964-74. In the course of analysis of eight mutants of the human cell line VA2B selected for their resistance to high concentrations of the complex I inhibitor rotenone, seven were found to be respiration deficient, and among these, six exhibited a specific defect of complex I. A detailed molecular analysis of the six complex I-deficient mutants revealed that two of them exhibited a frameshift mutation in the ND4 gene, in homoplasmic or in heteroplasmic form, resulting in the complete or partial loss, respectively, of the ND4 subunit; two other mutants exhibited a frameshift mutation in the ND5 gene, in near-homoplasmic or heteroplasmic form, resulting in the ND5 subunit being undetectable or strongly decreased, respectively. |
2(0,0,0,2) | Details |
| 17320357 | Park JS, Li YF, Bai Y: Yeast NDI1 improves oxidative phosphorylation capacity and increases protection against oxidative stress and cell death in cells carrying a Leber's hereditary optic neuropathy mutation. Biochim Biophys Acta. 2007 May;1772(5):533-42. Epub 2007 Jan 26. G11778A in the subunit ND4 gene of NADH dehydrogenase complex is the most common primary mutation found in Leber's hereditary optic neuropathy (LHON) patients. In transformant cell lines, LeNDI1-1 and -2, total and complex I-dependent respiration were fully restored and largely resistant to complex I inhibitor, rotenone, indicating a dominant role of NDI1 in the transfer of electrons in the host cells. |
1(0,0,0,1) | Details |
| 10491287 | Mills KI, Woodgate LJ, Gilkes AF, Walsh V, Sweeney MC, Brown G, Burnett AK: Inhibition of mitochondrial function in HL60 cells is associated with an increased apoptosis and expression of CD14. Biochem Biophys Res Commun. 1999 Sep 24;263(2):294-300. One of these clones was the mitochondrial gene NADH dehydrogenase subunit 4 (ND4) which showed a differential pattern of expression between the neutrophil and monocyte lineages. The potential of mitochondrial inhibitors to induce differentiation was investigated by treating the HL60 cells with either the NADH dehydrogenase inhibitor, Rotenone, the complex III inhibitor, Antimycin A, or the highly specific mitochondrial ATP-synthase inhibitor, Oligomycin. |
1(0,0,0,1) | Details |
| 7926004 | Degli Esposti M, Carelli V, Ghelli A, Ratta M, Crimi M, Sangiorgi S, Montagna P, Lenaz G, Lugaresi E, Cortelli P: Functional alterations of the mitochondrially encoded ND4 subunit associated with Leber's hereditary optic neuropathy. FEBS Lett. 1994 Oct 3;352(3):375-9. We report that this amino acid substitution alters the affinity of complex I for the substrate and induces resistance towards its potent inhibitor rotenone in mitochondria of LHON patients. |
3(0,0,0,3) | Details |
| 11695835 | Chomyn A: Mitochondrial genetic control of assembly and function of complex I in mammalian cells. J Bioenerg Biomembr. 2001 Jun;33(3):251-7. More recently, we carried out a biochemical, molecular, and cellular analysis of a mutation in the gene for one of these subunits, ND4, that causes Leber's hereditary optic neuropathy (LHON). Subsequently, we isolated several mutants affected in one or another of the mtDNA-encoded subunits of complex I by exposing established cell lines to high concentrations of rotenone. |
2(0,0,0,2) | Details |
| 1763894 | Larsson NG, Andersen O, Holme E, Oldfors A, Wahlstrom J: Leber's hereditary optic neuropathy and complex I deficiency in muscle. . Ann Neurol. 1991 Nov;30(5):701-8. We investigated a family with Leber's hereditary optic neuropathy in which affected individuals were homoplasmic for the point mutation of the NADH-dehydrogenase 4 gene of mitochondrial DNA, described by Wallace and colleagues in 1988. There was no decrease in complex I activity measured as ferricyanide reductase or rotenone-sensitive cytochrome c reductase activities. |
2(0,0,0,2) | Details |
| 10072046 | Carelli V, Ghelli A, Bucchi L, Montagna P, De Negri A, Leuzzi V, Carducci C, Lenaz G, Lugaresi E, Degli Esposti M: Biochemical features of mtDNA 14484 (ND6/M64V) point mutation associated with Leber's hereditary optic neuropathy. Ann Neurol. 1999 Mar;45(3):320-8. Our results suggest that both 14484 and 14459 mutations may affect amino acids forming the interaction site of product, and the 14484 mutation produces a biochemical defect resembling in part that already reported for the common 11778/ND4 LHON mutation. |
1(0,0,0,1) | Details |