title: Data on cytochrome c oxidase assembly in mice and human fibroblasts or tissues induced by SURF1 defect.
abstract: This paper describes data related to a research article entitled "Tissue- and species-specific differences in cytochrome c oxidase assembly induced by SURF1 defects" [1]. This paper includes data of the quantitative analysis of individual forms of respiratory chain complexes I, III and IV present in SURF1 knockout (SURF1 (-/-) ) and control (SURF1 (+/+) ) mouse fibroblasts and tissues and in fibroblasts of human control and patients with SURF1 gene mutation. Also it includes data demonstrating response of complex IV, cytochrome c oxidase (COX), to reversible inhibition of mitochondrial translation in SURF1 (-/-) mouse and SURF1 patient fibroblast cell lines.
authors.affiliation: Institute of Physiology of the Czech Academy of Sciences, Vídeňská 1083, Prague, Czech Republic.
authors.forename: Nikola
authors.initials: N
authors.lastname: Kovářová
authors.affiliation: Institute of Physiology of the Czech Academy of Sciences, Vídeňská 1083, Prague, Czech Republic.
authors.forename: Petr
authors.initials: P
authors.lastname: Pecina
authors.affiliation: Institute of Physiology of the Czech Academy of Sciences, Vídeňská 1083, Prague, Czech Republic.
authors.forename: Hana
authors.initials: H
authors.lastname: Nůsková
authors.affiliation: Institute of Physiology of the Czech Academy of Sciences, Vídeňská 1083, Prague, Czech Republic.
authors.forename: Marek
authors.initials: M
authors.lastname: Vrbacký
authors.affiliation: Molecular Neurogenetics Unit, Instituto Neurologico "C. Besta", via Temolo 4, 20126 Milan, Italy; MRC-Mitochondrial Biology Unit, Wellcome Trust MRC Bldg, Addenbrookes Hospital Hills Rd, Cambridge CB2 0XY, UK.
authors.forename: Massimo
authors.initials: M
authors.lastname: Zeviani
authors.affiliation: Institute of Physiology of the Czech Academy of Sciences, Vídeňská 1083, Prague, Czech Republic.
authors.forename: Tomáš
authors.initials: T
authors.lastname: Mráček
authors.affiliation: MRC-Mitochondrial Biology Unit, Wellcome Trust MRC Bldg, Addenbrookes Hospital Hills Rd, Cambridge CB2 0XY, UK.
authors.forename: Carlo
authors.initials: C
authors.lastname: Viscomi
authors.affiliation: Institute of Physiology of the Czech Academy of Sciences, Vídeňská 1083, Prague, Czech Republic.
authors.forename: Josef
authors.initials: J
authors.lastname: Houštěk
keywords: COX, Cytochrome c oxidase; Cytochrome c oxidase; DOX, doxycycline; Doxycycline; Knockout; Respiratory chain; SURF1