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Figure 3 | BMC Medical Genetics

Figure 3

From: Adenoviral-mediated correction of methylmalonyl-CoA mutase deficiency in murine fibroblasts and human hepatocytes

Figure 3

Expression and functional studies of methylmalonyl-CoA mutase in Mut murine embryonic fibroblasts. A. Western analysis of protein lysates from MEFs probed with methylmalonyl-CoA mutase antibody (78 kDa, labeled mutase) and cross-reactive beta-actin loading control (47 kDa, labeled actin). All wells had 10 micrograms of total protein loaded. Lane 1: Marker; Lane 2: Recombinant murine methylmalonyl-CoA mutase; Lane 3: Wild-type MEF; Lane 4: Murine methylmalonyl-CoA mutase knock-out MEFs; Lane 5: Adenoviral-corrected murine methylmalonyl-CoA mutase knock-out MEFs. B. Murine methylmalonyl-CoA mutase knock-out murine embryonic fibroblasts (uncorrected and corrected with adenovirus) were assayed for [1-14C] propionate incorporation over 18 hours. Activity was normalized to total protein content of the extracts. The samples were analyzed in triplicate. The bars around the average represent +/- one standard deviation.

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