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Tissue- and species-specific differences in cytochrome c oxidase assembly induced by SURF1 defects
Mitochondrial protein SURF1 is a specific assembly factor of cytochrome c oxidase (COX), but its function is poorly understood. SURF1 gene mutations cause a severe COX deficiency manifesting as the Leigh syndrome in humans, whereas in mice SURF1(−/−) knockout leads only to a mild COX defect. We used...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier Pub. Co
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4793088/ https://www.ncbi.nlm.nih.gov/pubmed/26804654 http://dx.doi.org/10.1016/j.bbadis.2016.01.007 |