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. 2012 Apr 25;287(24):20652–20663. doi: 10.1074/jbc.M111.327601

FIGURE 3.

FIGURE 3.

B2 SINE insertion into Ndufs4 results in isolated CI deficiency in all tissues tested. A, RT-PCR showing truncated Ndufs4 transcripts in whole brain RNA of Ndufs4fky/fky mice; wild type transcript variant (V1) was identified through the sequencing of clones; the schematic on the right describes the different amplicons visible on the gel; white arrows indicate the locations of stop codons. B, NDUFS4 protein was not detected in whole brain, heart, liver, and skeletal muscle of Ndufs4fky/fky mice via Western blot. C, CI activity, normalized against citrate synthase (CS), was reduced in all tissues tested of Ndufs4fky/fky mice (n = 3–5). D, mitochondrial CII, CIII, and CIV activities were normal in brain tissues of Ndufs4fky/fky mice (n = 4). E, ATP synthesis capacity of heart and brain mitochondria was determined as the CI-dependent ATP production (with substrate combination malate and glutamate) normalized against CII-dependent ATP production (with substrate succinate in the presence of CI inhibitor rotenone) and is shown as a percentage of control mitochondria (age range P30–P41, heart n = 13, brain n = 5). C and D, black bars, wild type mice; white bars, Ndufs4fky/fky mice. E, control, Ndufs4+/f+ and Ndufs4+/fky; fky/fky, Ndufs4fky/fky. *, p < 0.05; **, p < 0.01; ***, p < 0.005; #, p < 0.001; ##, p < 0.0005; ###, p < 0.0001; ####, p < 0.00005. Error bars, S.E.