Skip to main content
. 2018 Mar 22;102(4):685–695. doi: 10.1016/j.ajhg.2018.02.012

Figure 5.

Figure 5

Pulse Labeling of Mitochondrial Translation Products and OXPHOS Assembly Analysis

(A) Pulse labeling of mitochondrial translation products in control (C1–C3), S1, S2, and S3 fibroblasts shows decreased mitochondrial protein synthesis in all subjects’ cell lines. Coomassie staining of the gels was used for the assessment of loading.

(B) BN-PAGE analysis of OXPHOS assembly in control, S1, S2, and S3 fibroblasts shows decreased amounts of fully assembled OXPHOS complexes I and IV in the fibroblasts from the MRPS2- and MRPS22-deficient subjects. Subcomplexes of complex V accumulate in subjects’ fibroblasts and are absent in the control cells.