10.6084/m9.figshare.c.3747815_D4.v1 Saara Tegelberg Saara Tegelberg Nikica Tomašić Nikica Tomašić Jukka Kallijärvi Jukka Kallijärvi Janne Purhonen Janne Purhonen Eskil Elmér Eskil Elmér Eva Lindberg Eva Lindberg David Nord David Nord Maria Soller Maria Soller Nicole Lesko Nicole Lesko Anna Wedell Anna Wedell Helene Bruhn Helene Bruhn Christoph Freyer Christoph Freyer Henrik Stranneheim Henrik Stranneheim Rolf Wibom Rolf Wibom Inger Nennesmo Inger Nennesmo Anna Wredenberg Anna Wredenberg Erik Eklund Erik Eklund Vineta Fellman Vineta Fellman Additional file 4: Figure S3. of Respiratory chain complex III deficiency due to mutated BCS1L: a novel phenotype with encephalomyopathy, partially phenocopied in a Bcs1l mutant mouse model Springer Nature 2017 Mitochondrial disorder Respiratory chain Respirometry Assembly factors Blue native gel electrophoresis Encephalopathy Hepatopathy Microglia Barrel cortex 2017-04-20 05:00:00 Journal contribution https://springernature.figshare.com/articles/journal_contribution/Additional_file_4_Figure_S3_of_Respiratory_chain_complex_III_deficiency_due_to_mutated_BCS1L_a_novel_phenotype_with_encephalomyopathy_partially_phenocopied_in_a_Bcs1l_mutant_mouse_model/4897988 BNGE with immunblotting. The samples were run on two gels in quadruplicate. (A). The gel was stained with commassie blue after blotting to PVDF membrane to show that the loading was similar; the first (lanes 2-5) and second (lanes 7-10) loading of the samples with the ladder (lanes 1 and 6) are shown. The molecular weights of the ladder markers are indicated. (B). For the upper blot the CORE1 and RISP antibodies were used, for the second blot the BCS1L antibody and the combination of CI NDUFVI (to detect the subunit assembled at the final stage), CIV Va, and CII 30kD were used, respectively. The first blot was stripped and thereafter the antibodies against CIV COX and CI NDUFA9 were probed (remnants of the CORE1 and RISP bands can be seen). Despite weaker bands in the patient (lanes 1 and 6) the decrease in BCS1L and RISP is recognizable. (PDF 2082 kb)