Related Content
Search Google Scholar for:
|
|
Science 31 October 1986: Vol. 234. no. 4776, pp. 614 - 618 DOI: 10.1126/science.3764430
|
|
Articles
Science, Vol 234, Issue 4776, 614-618
Copyright © 1986 by American Association for the Advancement of Science
URF6, last unidentified reading frame of human mtDNA, codes for an NADH dehydrogenase subunit
A Chomyn,
MW Cleeter,
CI Ragan,
M Riley,
RF Doolittle,
and
G Attardi
The polypeptide encoded in URF6, the last unassigned reading frame of human mitochondrial DNA, has been identified with antibodies to peptides predicted from the DNA sequence. Antibodies prepared against highly purified respiratory chain NADH dehydrogenase from beef heart or against the cytoplasmically synthesized 49-kilodalton iron-sulfur subunit isolated from this enzyme complex, when added to a deoxycholate or a Triton X-100 mitochondrial lysate of HeLa cells, specifically precipitated the URF6 product together with the six other URF products previously identified as subunits of NADH dehydrogenase. These results strongly point to the URF6 product as being another subunit of this enzyme complex. Thus, almost 60% of the protein coding capacity of mammalian mitochondrial DNA is utilized for the assembly of the first enzyme complex of the respiratory chain. The absence of such information in yeast mitochondrial DNA dramatizes the variability in gene content of different mitochondrial genomes.
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
- Characterization of the NuoM (ND4) Subunit in Escherichia coli NDH-1: CONSERVED CHARGED RESIDUES ESSENTIAL FOR ENERGY-COUPLED ACTIVITIES.
- J. Torres-Bacete, E. Nakamaru-Ogiso, A. Matsuno-Yagi, and T. Yagi (2007)
J. Biol. Chem.
282, 36914-36922
| Abstract »
| Full Text »
| PDF »
- Selective and Nonselective Packaging of Cellular RNAs in Retrovirus Particles.
- S. J. Rulli Jr., C. S. Hibbert, J. Mirro, T. Pederson, S. Biswal, and A. Rein (2007)
J. Virol.
81, 6623-6631
| Abstract »
| Full Text »
| PDF »
- Nuclear Suppression of Mitochondrial Defects in Cells without the ND6 Subunit.
- J.-H. Deng, Y. Li, J. S. Park, J. Wu, P. Hu, J. Lechleiter, and Y. Bai (2006)
Mol. Cell. Biol.
26, 1077-1086
| Abstract »
| Full Text »
| PDF »
- The Post-translational Modifications of the Nuclear Encoded Subunits of Complex I from Bovine Heart Mitochondria.
- J. Carroll, I. M. Fearnley, J. M. Skehel, M. J. Runswick, R. J. Shannon, J. Hirst, and J. E. Walker (2005)
Mol. Cell. Proteomics
4, 693-699
| Abstract »
| Full Text »
| PDF »
- Mitochondrial tRNA 3' end metabolism and human disease.
- L. Levinger, M. Morl, and C. Florentz (2004)
Nucleic Acids Res.
32, 5430-5441
| Abstract »
| Full Text »
| PDF »
- Functional Roles of Four Conserved Charged Residues in the Membrane Domain Subunit NuoA of the Proton-translocating NADH-Quinone Oxidoreductase from Escherichia coli.
- M.-C. Kao, S. Di Bernardo, M. Perego, E. Nakamaru-Ogiso, A. Matsuno-Yagi, and T. Yagi (2004)
J. Biol. Chem.
279, 32360-32366
| Abstract »
| Full Text »
| PDF »
- Pathological Mutations of the Human NDUFS4 Gene of the 18-kDa (AQDQ) Subunit of Complex I Affect the Expression of the Protein and the Assembly and Function of the Complex.
- S. Scacco, V. Petruzzella, S. Budde, R. Vergari, R. Tamborra, D. Panelli, L. P. van den Heuvel, J. A. Smeitink, and S. Papa (2003)
J. Biol. Chem.
278, 44161-44167
| Abstract »
| Full Text »
| PDF »
- The enigmatic mitochondrial ORF ymf39 codes for ATP synthase chain b.
- G. Burger, B. F. Lang, H.-P. Braun, and S. Marx (2003)
Nucleic Acids Res.
31, 2353-2360
| Abstract »
| Full Text »
| PDF »
- Analysis of the Subunit Composition of Complex I from Bovine Heart Mitochondria.
- J. Carroll, I. M. Fearnley, R. J. Shannon, J. Hirst, and J. E. Walker (2003)
Mol. Cell. Proteomics
2, 117-126
| Abstract »
| Full Text »
| PDF »
- Definition of the Nuclear Encoded Protein Composition of Bovine Heart Mitochondrial Complex I. IDENTIFICATION OF TWO NEW SUBUNITS.
- J. Carroll, R. J. Shannon, I. M. Fearnley, J. E. Walker, and J. Hirst (2002)
J. Biol. Chem.
277, 50311-50317
| Abstract »
| Full Text »
| PDF »
- Lack of Complex I Activity in Human Cells Carrying a Mutation in MtDNA-encoded ND4 Subunit Is Corrected by the Saccharomyces cerevisiae NADH-Quinone Oxidoreductase (NDI1) Gene.
- Y. Bai, P. Hajek, A. Chomyn, E. Chan, B. B. Seo, A. Matsuno-Yagi, T. Yagi, and G. Attardi (2001)
J. Biol. Chem.
276, 38808-38813
| Abstract »
| Full Text »
| PDF »
- A nonsense mutation in the NDUFS4 gene encoding the 18 kDa (AQDQ) subunit of complex I abolishes assembly and activity of the complex in a patient with Leigh-like syndrome.
- V. Petruzzella, R. Vergari, I. Puzziferri, D. Boffoli, E. Lamantea, M. Zeviani, and S. Papa (2001)
Hum. Mol. Genet.
10, 529-535
| Abstract »
| Full Text »
| PDF »
- Characterization of control and immobilized skeletal muscle: an overview from genetic engineering.
- J. ST-AMAND, K. OKAMURA, K. MATSUMOTO, S. SHIMIZU, and Y. SOGAWA (2001)
FASEB J
15, 684-692
| Abstract »
| Full Text »
| PDF »
- Complex I Impairment, Respiratory Compensations, and Photosynthetic Decrease in Nuclear and Mitochondrial Male Sterile Mutants of Nicotiana sylvestris.
- M. Sabar, R. De Paepe, and Y. de Kouchkovsky (2000)
Plant Physiology
124, 1239-1250
| Abstract »
| Full Text »
- Identification and Characterization of Human Mitochondrial Tryptophanyl-tRNA Synthetase.
- R. Jorgensen, T. M. M. Sogaard, A. B. Rossing, P. M. Martensen, and J. Justesen (2000)
J. Biol. Chem.
275, 16820-16826
| Abstract »
| Full Text »
| PDF »
- Titrating the Effects of Mitochondrial Complex I Impairment in the Cell Physiology.
- A. Barrientos and C. T. Moraes (1999)
J. Biol. Chem.
274, 16188-16197
| Abstract »
| Full Text »
| PDF »
- Mitochondrial protein synthesis in rainbow trout (Oncorhynchus mykiss) heart is enhanced in sexually mature males but impaired by low temperature.
- J. West and W. Driedzic (1999)
J. Exp. Biol.
202, 2359-2369
| Abstract »
| PDF »
- Molecular remedy of complex I defects: Rotenone-insensitive internal NADH-quinone oxidoreductase of Saccharomyces cerevisiae mitochondria restores the NADH oxidase activity of complex I-deficient mammalian cells.
- B. B. Seo, T. Kitajima-Ihara, E. K. L. Chan, I. E. Scheffler, A. Matsuno-Yagi, and T. Yagi (1998)
PNAS
95, 9167-9171
| Abstract »
| Full Text »
| PDF »
- Human Xenomitochondrial Cybrids. CELLULAR MODELS OF MITOCHONDRIAL COMPLEX I DEFICIENCY.
- A. Barrientos, L. Kenyon, and C. T. Moraes (1998)
J. Biol. Chem.
273, 14210-14217
| Abstract »
| Full Text »
| PDF »
- Lack of mitochondrial and nuclear-encoded subunits of complex I and alteration of the respiratory chain in Nicotiana sylvestris mitochondrial deletion mutants.
- S. Gutierres, M. Sabar, C. Lelandais, P. Chetrit, P. Diolez, H. Degand, M. Boutry, F. Vedel, Y. de Kouchkovsky, and R. De Paepe (1997)
PNAS
94, 3436-3441
| Abstract »
| Full Text »
| PDF »
- Respiration and Growth Defects in Transmitochondrial Cell Lines Carrying the 11778 Mutation Associated with Leber's Hereditary Optic Neuropathy.
- G. Hofhaus, D. R. Johns, O. Hurko, G. Attardi, and A. Chomyn (1996)
J. Biol. Chem.
271, 13155-13161
| Abstract »
| Full Text »
| PDF »
- Mitochondrial DNA mutation associated with Leber's hereditary optic neuropathy.
- D. Wallace, G Singh, M. Lott, J. Hodge, T. Schurr, A. Lezza, L. Elsas 2nd, and E. Nikoskelainen (1988)
Science
242, 1427-1430
| Abstract »
| PDF »
- Interaction of Mammalian Mitochondrial Ribosomes with the Inner Membrane.
- M. Liu and L. Spremulli (2000)
J. Biol. Chem.
275, 29400-29406
| Abstract »
| Full Text »
| PDF »
- Use of the NADH-Quinone Oxidoreductase (NDI1) Gene of Saccharomyces cerevisiae as a Possible Cure for Complex I Defects in Human Cells.
- B. B. Seo, J. Wang, T. R. Flotte, T. Yagi, and A. Matsuno-Yagi (2000)
J. Biol. Chem.
275, 37774-37778
| Abstract »
| Full Text »
| PDF »
|
|