Figure 4.

Mitochondrial morphology in UVRAG-deficient muscle. (A) Representative TEM images of intermyofibrillar (IMF) mitochondria and quantification (B) of mitochondrial morphology from the extensor digitorum longus (EDL) at 6 months of age (n = 3). Swollen mitochondrion is indicated by arrows. Scale bar, 200 nm. (C) Immunoblots (left) and quantification (right) of total- and phospho-Drp1 from quadriceps of thee 6-month-old mice (n = 5). GAPDH was used as a loading control. (D) Representative immunofluorescent images (left) of MitoTracker (red), phospho-Drp1 (green), DAPI (blue), and quantification of green fluorescence intensity (right) from C2C12 myotubes transfected with siUVRAG or scramble (Scr) knockdown (n = 3). Scale bar, 7 μm. (E) ImageJ quantitation of mitochondrial length from MitoTracker-stained C2C12 myotubes transfected with siUVRAG or scramble (Scr) knockdown (n = 5). (F) Representative immunofluorescent images (left) of MitoTracker (red) and LysoTracker (green) and quantification of colocalization (right) from C2C12 myotubes transfected with siUVRAG or scramble (Scr) knockdown (n = 3). Scale bar, 5 μm. (G) Relative expression of genes related to mitophagy from quadriceps of the M-UVRAG^−/− mice relative to the M-UVRAG^+/+ mice at 6 months of age (n = 4–6). Data shown are mean ± S.E.M. ∗p < 0.05, ∗∗p < 0.01, and ∗∗∗p < 0.001 using Student's t test.