Figure 2.

MtDNA depletion in TWINKLE knockout mice. (A) Southern blot analysis of mtDNA levels from wild-type (L/L) and tissue-specific knockout mice (L/L, cre) at different ages. The nuclear 18S rRNA gene was used as a loading control. (B) Southern blot quantification: ratio of the mtDNA signal to 18S DNA loading control from four independent experiments. Error bars represent the SEM. (C) Long-extension PCR of heart (H) skeletal muscle (M) isolated total DNA from 12-week-old wild-type (L/L) and tissue-specific knockout mice (L/L, cre) using primers P0 and P1. A vector containing whole mtDNA was used as a control (V).