000			02015na a2200265 4500
999			_c5904 _d5904
003			PC5904
005			20210625062803.0
008			130622s2013 xxx||||| |||| 00| 0 eng d
040			_cH12O
041			_aeng
100			_aFernández Moreno, Miguel Ángel _9860 _eInstituto de Investigación i+12
100			_9862 _aGaresse, Rafael _eInstituto de Investigación i+12
245	0	0	_ahCOA3 Stabilizes Cytochrome c Oxidase 1 (COX1) and Promotes Cytochrome c Oxidase Assembly in Human Mitochondria. _h[artículo]
260			_bJournal of Biological Chemistry, _c2013
300			_a288(12):8321-31.
500			_aFormato Vancouver: Clemente P, Peralta S, Cruz-Bermúdez A, Echevarría L, Fontanesi F, Barrientos A et al. hCOA3 stabilizes cytochrome c oxidase 1 (COX1) and promotes cytochrome c oxidase assembly in human mitochondria. J Biol Chem. 2013 Mar 22;288(12):8321-31.
501			_aPMID: 23362268
504			_aContiene 45 referencias
520			_aCytochrome c oxidase (COX) or complex IV of the mitochondrial respiratory chain plays a fundamental role in energy production of aerobic cells. In humans, COX deficiency is the most frequent cause of mitochondrial encephalomyopathies. Human COX is composed of 13 subunits of dual genetic origin, whose assembly requires an increasing number of nuclear-encoded accessory proteins known as assembly factors. Here, we have identified and characterized human CCDC56, an 11.7-kDa mitochondrial transmembrane protein, as a new factor essential for COX biogenesis. CCDC56 shares sequence similarity with the yeast COX assembly factor Coa3 and was termed hCOA3. hCOA3-silenced cells display a severe COX functional alteration owing to a decreased stability of newly synthesized COX1 and an impairment in the holoenzyme assembly process. We show that hCOA3 physically interacts with both the mitochondrial translation machinery and COX structural subunits. We conclude that hCOA3 stabilizes COX1 co-translationally and promotes its assembly with COX partner subunits. Finally, our results identify hCOA3 as a new candidate when screening for genes responsible for mitochondrial diseases associated with COX deficiency.
710			_9625 _aInstituto de Investigación imas12
856			_uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3605650/pdf/zbc8321.pdf _yAcceso libre
942			_n0 _2ddc _cART