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. 2022 Jul 15;132(14):e157504. doi: 10.1172/JCI157504

Figure 4. Intracristal inclusions are characteristic of affected C10 G58R patient and mouse muscle.

Figure 4

(A) Intramitochondrial inclusions in muscles of patients with the G58R mutation (arrowheads). Scale bar: 1 μm. The “UK family II-2” image was reproduced with permission from The Lancet (ref. 31), and the “US family patient” image was reproduced with permission from Muscle & Nerve (ref. 26). (B) TEM of 28-week-old C10WT and C10G58R mouse hearts. Arrowheads indicate intramitochondrial inclusions (representative of ≥15 fields in 2 biological replicates). (C) TEM of tibialis from 14-week-old C10WT and C10G58R mice on the OMA1+/– background. Arrowheads indicate intramitochondrial inclusions (representative of ≥10 fields in 1 biological replicate). Scale bars: 1 μm and 400 nm (B and C). (D) Serial TEM following an intramitochondrial inclusion containing a membranous vesicle within (representative of ≥6 mitochondria from 1 biological replicate). Scale bar: 400 nm. (E) Top: TEM of an ultrathin section of a 33-week-old C10G58R mouse heart showing cristal dilation, a single-membraned intracristal vesicle (arrowhead), and a double-membraned intracristal vesicle (arrow). Bottom: Higher-magnification (×3.5) image showing continuity with the IMM, indicated by arrowheads. Right: Sketch and labels for the image on the left (representative of ≥10 mitochondria in 1 biological replicate). (F) TEM of an ultrathin section of a 33-week-old C10G58R mouse heart showing inner membrane active budding events (arrows) and completed budding (arrowheads) (representative of ≥5 mitochondria in 1 biological replicate). Scale bar: 100 μm.