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.

Institute of Physiology of the Czech Academy of Sciences Vídeňská 1083 Prague Czech Republic

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

MRC Mitochondrial Biology Unit Wellcome Trust MRC Bldg Addenbrookes Hospital Hills Rd Cambridge CB2 0XY UK

Zobrazit více v PubMed

Kovářová N., Pecina P., Nůsková H., Vrbacký M., Zeviani M., Mráček T., Viscomi C., Houštěk J. Tissue- and species-specific differences in cytochrome c oxidase assembly induced by SURF1 defects. Biochim. Biophys. Acta. 1862;4(2016):705–715. PubMed PMC

Dell׳Agnello C., Leo S., Agostino A., Szabadkai G., Tiveron C., Zulian A., Prelle A., Roubertoux P., Rizzuto R., Zeviani M. Increased longevity and refractoriness to Ca(2+)-dependent neurodegeneration in Surf1 knockout mice. Hum. Mol. Genet. 2007;16(4):431–444. PubMed

Pecina P., Čapková M., Chowdhury S.K., Drahota Z., Dubot A., Vojtíšková A., Hansíková H., Houšťková H., Zeman J., Godinot C., Houštěk J. Functional alteration of cytochrome c oxidase by SURF1 mutations in Leigh syndrome. Biochim. Biophys. Acta. 1639;1(2003):53–63. PubMed

Bentlage H.A., Wendel U., Schägger H., ter Laak H.J., Janssen A.J., Trijbels J.M. Lethal infantile mitochondrial disease with isolated complex I deficiency in fibroblasts but with combined complex I and IV deficiencies in muscle. Neurology. 1996;47(1):243–248. PubMed

Wittig I., Braun H.P., Schägger H. Blue native PAGE. Nat. Protoc. 2006;1(1):418–428. PubMed

Bradford M.M. A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein–dye binding. Anal. Biochem. 1976;72:248–254. PubMed

Schägger H., von Jagow G. Blue native electrophoresis for isolation of membrane protein complexes in enzymatically active form. Anal. Biochem. 1991;199(2):223–231. PubMed

Schägger H., von Jagow G. Tricine-sodium dodecyl sulfate-polyacrylamide gel electrophoresis for the separation of proteins in the range from 1 to 100 kDa. Anal. Biochem. 1987;166(2):368–379. PubMed

Moreno-Lastres D., Fontanesi F., García-Consuegra I., Martín M.A., Arenas J., Barrientos A., Ugalde C. Mitochondrial complex I plays an essential role in human respirasome assembly. Cell. Metab. 2012;15(3):324–335. PubMed PMC