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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...

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Detalles Bibliográficos
Autores principales: Kovářová, Nikola, Pecina, Petr, Nůsková, Hana, Vrbacký, Marek, Zeviani, Massimo, Mráček, Tomáš, Viscomi, Carlo, Houštěk, Josef
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier Pub. Co 2016
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