SDH mutation-induced saccharopine accumulation causes mitochondrial damage and functional loss and shortens C. elegans body length. (A and B) Fold change of saccharopine (A) and lysine (B) levels in animals with the indicated genotypes. Data (mean ± SEM) were derived from three independent experiments as shown in Fig. S2 and normalized to saccharopine or lysine intensities in N2 animals. (C) Images (left) and quantification (right) of the suppression of aass-1(yq170) mitochondrial defects by slc-25A29(yq276) mutation. 90 animals or more were scored for each genotype. Bars, 5 µm. (D) TEM images of mitochondria in the hypodermis in adult animals with the indicated genotypes. Boxed regions showing crista structures are magnified (3×) and shown in the bottom left corner in each image. Yellow arrows indicate mitochondrial fusion, and red arrowheads indicate broken mitochondria. Bars, 1 µm. (E) Representative images of 3D reconstructed mitochondria in the hypodermis of N2 (top) and aass-1(yq170) (bottom) animals. Bars, 1 µm. (F) Fold change of ATP levels in the hypodermis of animals with the indicated genotypes. Data (mean ± SEM) are from three independent experiments and normalized to the ATP levels in N2 animals. (G and H) Quantification of Mito-CMXROS (G) and Mito-cpYFP (H) intensities in adult animals with the indicated genotypes. 10 synchronized animals were analyzed for each genotype. (I) Survival curves of C. elegans animals with indicated genotypes. 100 animals were analyzed for each genotype. (J) Analysis of body lengths of adult animals with the indicated genotypes. 10 synchronized animals of each genotype were analyzed at every time point. For all quantifications, *, P < 0.05; **, P < 0.01; and ***, P < 0.001. Error bars represent SEM.