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Fig. 6 | Orphanet Journal of Rare Diseases

Fig. 6

From: Clinical and biochemical characterization of four patients with mutations in ECHS1

Fig. 6

Pyruvate dehydrogenase subunits in tissue samples. Levels of subunits of pyruvate dehydrogenase are shown in liver (a), muscle (b) and fibroblasts (c). Five control samples are shown followed by samples of patients 1 and 2. Decreased levels of the E2 subunit are shown when probed with a pyruvate dehydrogenase antibody cocktail, as well as with an antibody specific to E2 and E3-binding protein. GAPDH, porin and citrate synthase are shown as loading controls

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