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. 2012 Oct 5;91(4):737–743. doi: 10.1016/j.ajhg.2012.08.020

Figure 4.

Figure 4

Rescue of the Biochemical Phenotype by RMND1 Expression and Recapitulation of the Defect by siRNA-Mediated Knockdown of the Protein

(A) BN-PAGE analysis of controls and subject fibroblasts expressing RMND1 from a retroviral vector (pBABE). Each of the five OXPHOS complexes (I–V) was visualized with a subunit-specific antibody.

(B) Immunoblot analysis of the same samples as in (A) for expression of RMND1, individual structural subunits of the five OXPHOS complexes, and two mitochondrial ribosomal subunits. The 70 kDa subunit of complex II (SDHA) was used as a loading control.

(C) Analysis of mitochondrial translation products in control and subject fibroblasts transduced with retroviral vectors expressing RMND1-HA or RMND1-flag.

(D) Stealth RNAi-mediated knockdown of RMND1. The upper panel shows the level of knockdown of RMND1 on an immunoblot with VDAC1 (porin) as a loading control. The bottom panel shows the BN-PAGE analysis in the control, RMND1 knockdown, and subject cells.