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

Figure 6.

Figure 6

Amounts of Wild-Type MRPS2 in S1 and S2 Fibroblasts

(A) Immunoblot analysis of whole-cell extracts from control (C), S1, and S2 cell lines transduced with a lentivirus expressing either wild-type MRPS2 (+MRPS2) or green fluorescent protein (+GFP) as a negative control. The presence of wild-type MRPS2 leads to increased amounts of MRPS5 and MRPS18B, as well as NDUFB8 and NDUFA13 from complex I and COXI and COXIV from complex IV. MRPS2 was detected with a specific antiserum that does not detect the endogenous MRPS2 in whole-cell extracts. SDHA was used as a loading control for each separate gel.

(B) Pulse labeling of mitochondrial translation products demonstrates that the presence of wild-type MRPS2 partially restores mitochondrial translation in S1 and S2 fibroblasts. The gel was stained with Coomassie colloidal dye for the assessment of loading.

(C) BN-PAGE analysis of OXPHOS assembly in C, S1, and S2 fibroblasts transfected with either GFP- or wild-type-MRPS2-expressing lentivirus shows partial restoration of OXPHOS complex assembly in subject cells expressing MRPS2.