Figure 6.

TWINKLE overexpression increases mtDNA in BAC transgenic mice. (A) Western blot analysis of the steady-state levels of various mitochondrial proteins from isolated liver and heart mitochondria of wild-type (+/+) and transgenic (+/T) mice. (B) Southern blot analysis and quantification of mtDNA levels in total DNA extracts from liver and heart from wild-type (+/+) and transgenic (+/T) mice. The nuclear 18S rRNA gene was used as a loading control. Error bars represent SEM. (C) Western blot analysis of the steady-state levels of TWINKLE protein from isolated heart, liver and kidney mitochondria from TFAM knockout mice (L/L, cre) and their controls (L/L) and from Tfam PAC transgenic animals (+/T) and their controls (+/+). VDAC levels were used as a loading control.