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Figure 3 | Orphanet Journal of Rare Diseases

Figure 3

From: HIBCH mutations can cause Leigh-like disease with combined deficiency of multiple mitochondrial respiratory chain enzymes and pyruvate dehydrogenase

Figure 3

Cytochrome oxidase immunocytochemistry in cultured skin fibroblasts. Expression of complex IV subunit 1 (MTCO1) in control and Patient 3 fibroblasts. Cells were cultured in the presence of 5 μM MitoTracker® Red CM-H2-XRos to label mitochondria red, followed by green immunostaining with anti-MTCO1 antibodies and blue nuclear counterstaining with DAPI. (a) Pseudo-coloured fluorescent micrographs. (b) Relative cellular expression levels of MTCO1 in Control (blue bars) and Patient (red bars) cells. The mean grey levels were measured for both the green and red images of 3631 control and 2184 patient cells, and the ratio of the two signals was calculated for each cell. The histogram reveals the frequency of cells with a particular MTCO1/MitoTracker ratio at intervals of 0.025. The total number of cells per culture is 100%.

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172