Fig. 4.

ECHS1 KO cells have decreased OXPHOS protein steady‐state levels. (A) Steady‐state levels of NDUFB8 and MT‐CO2 were decreased in ECHS1 KO mitochondria, whereas levels of the voltage‐dependent anion‐selective channel protein 1 (VDAC1) were increased. (B) Steady‐state levels of complex I (CI), the complex III dimer (CIII₂), complex IV (CIV), complex V (CV), the CIII₂/CIV supercomplex and the CI/CIII₂/CIV supercomplex were all reduced in ECHS1 KO mitochondria compared to control (CON) mitochondria. Data shown as mean ± SD, n = 3. *P < 0.05, **P < 0.01 and ***P < 0.001 compared to control values (Student's two‐tailed t‐test).