Fig. 1.

Western blot analysis of TFPα and TFPβ in patient fibroblasts.

A. TFPα and TFPβ expression was decreased in patient 1 compared to their expression in control cells. B. Decreased expression of TFPα and TFPβ was observed in patient 2 compared to their expression in control cells. TFPα, α-subunit of TFP; TFPβ, β-subunit of TFP; VLCAD, very-long-chain acyl-CoA dehydrogenase; MCAD, medium-chain acyl-CoA dehydrogenase; ETFα, α-subunit of electron transfer flavoprotein; ETFβ, β-subunit of electron transfer flavoprotein; TFP, mitochondrial trifunctional protein.