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. 2015 Oct 5;112(42):E5689–E5698. doi: 10.1073/pnas.1506129112

Fig. 1.

Fig. 1.

Scheme of transgenic mice generation. (A) Mitochondrially encoded human mutant G11778A ND4 fused to FLAG (mutND4FLAG) and mitochondrially encoded mCherry were inserted into the scAAV backbone under the control of the human mitochondrial HSP. The stop codon AGA separated the two genes. (B–D)The construct was packaged in mitochondrially targeted cox8 MTS-scAAV (B) and was microinjected into fertilized eggs (C and D). (E) Offspring were screened for mCherry by CLSO (Inset). (F–H) Females with higher mCherry expression (F) were backcrossed with wild-type C57BL/6 males (G), generating mutant ND4 mice (H). (I)The transgenic mouse (arrow) was larger than a control mouse. (J–O) CLSO imaging of the optic nerve head (onh) and retinal nerve fiber layer (arrows) shows red fluorescent particles (arrows) in the retina of first-generation LHON mito-mice (F0) at 2 mo (2m) (J and K) and 11 mo (11m) (L) after birth and in their F1 progeny at 2m (M and N) and 7m (O) after birth. bGH-Poly(A), bovine growth hormone polyadenylation sequence; iTR, inverted terminal repeats.