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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.

SUBMITTER: Kovarova N 

PROVIDER: S-EPMC4927972 | biostudies-literature | 2016 Jun

REPOSITORIES: biostudies-literature

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Data on cytochrome c oxidase assembly in mice and human fibroblasts or tissues induced by SURF1 defect.

Kovářová Nikola N   Pecina Petr P   Nůsková Hana H   Vrbacký Marek M   Zeviani Massimo M   Mráček Tomáš T   Viscomi Carlo C   Houštěk Josef J  

Data in brief 20160326


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 r  ...[more]

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