Figure 2.
Mitochondrial length analysis using TMRM staining and confocal microscopy. (A) TMRM labelled mitochondria in Patients 1–3 fibroblasts (top), transduced with wild-type STAT2 (middle), and DRP1−, STAT1− and healthy control (HC) cells (bottom); all cells at 70% confluency. Minus sign represents autosomal dominant mutation. (B) TMRM labelled mitochondria in SHSY5Y cells, wild-type (left) and STAT2 knockout (right). (C) ImageJ quantitation of mitochondrial perimeter length in fibroblasts (top) and SHSY5Y cells (bottom). **P < 0.005.