Rescue of Protein Lipoylation in Affected Individuals with LIPT2 Deficiency
Fibroblasts from affected individuals were transfected with wild-type LIPT2. Immunostaining analysis of protein-bound lipoic acid showed increase of protein lipoylation after transfection. The following antibodies and reagents were used: rabbit anti-LIPT2 (Novus Biological, ImmunoFluorescence [IF]: 1:100), rabbit anti-lipoic acid (Abcam cat# ab58724, RRID: AB_880635; 1:1,000), and DAPI 300 nM in PBS (D1306, Molecular Probes).