NDUFS6 mutations are a novel cause of lethal neonatal mitochondrial complex I deficiency
J. Clin. Invest. Denise M. Kirby, et al. 114:837 doi:10.1172/JCI20683 [Go to this article.]

Figure 2
Immunoblot analysis and quantitation of complexes I and II from fibroblasts of patients A_J and controls (N) following separation by BN-PAGE. (A) Fully assembled complex I detected by an antibody to the 39-kDa subunit is at approximately 900 kDa. Partially assembled complex I detected by the same antibody is at approximately 750 kDa, and fully assembled complex II detected by an antibody to the 70-kDa subunit is at approximately 130 kDa. (B) Histogram showing percentage fully (white bars) and partially (gray bars) assembled complex I relative to fully assembled complex II in primary fibroblasts. The error bar represents the observed range for 2 different control cell lines.