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

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)