Figure 6
SLP2/PHB2 aggregates with increased cell death in spinal motor neurons of Chchd10S59L/+ mice. (A) Immunolabelling with antibodies against SLP2 and PHB2 in MNs on lumbar spinal cord sections from Chchd10S59L/+ mice (S59L/+) and control littermates (+/+) at end-stage. Scale bar = 10 µm. (B) Lumbar spinal cord sections visualizing spinal MNs from Chchd10S59L/+ mice (S59L/+) and control littermates (+/+) at end-stage, immunolabelled with antibodies against SLP2, PHB2 and Vimentin. Scale bar = 10 µm. (C) Lumbar spinal cord sections from Chchd10S59L/+ mice (S59L/+) and control littermates (+/+) at end-stage. Immunolabelling was performed with antibodies against CHCHD10, PHB2 and Vimentin. Scale bar = 10 µm. (D) Lumbar spinal cord sections from Chchd10S59L/+ mice (S59L/+) and control littermates (+/+) at end-stage, immunolabelled with antibodies against CHCHD10, SLP2 and Vimentin. Scale bar = 10 µm. (E) Lumbar spinal cord sections from Chchd10S59L/+ mice (S59L/+) at end-stage. Left, TUNEL assay was combined with immunolabelling by using antibodies against SLP2 and Vimentin. Arrows indicate apoptotic (TUNEL+/DAPI+/SLP2+/Vimentin+) neurons. Asterix indicates a DAPI+/TUNEL−/SLP2−/Vimentin− neuron. Right, Quantification of SLP2+/Vimentin+ neurons (Supplementary Fig. 6C) displaying TUNEL-positive signal. n = 3 independent experiments from three Chchd10S59L/+ mice and three control littermates. A total of 96–103 SLP2+/Vimentin+ MNs from S59L/+ mice or control littermates was analysed. Scale bar = 20 µm.

SLP2/PHB2 aggregates with increased cell death in spinal motor neurons of Chchd10S59L/+ mice. (A) Immunolabelling with antibodies against SLP2 and PHB2 in MNs on lumbar spinal cord sections from Chchd10S59L/+ mice (S59L/+) and control littermates (+/+) at end-stage. Scale bar = 10 µm. (B) Lumbar spinal cord sections visualizing spinal MNs from Chchd10S59L/+ mice (S59L/+) and control littermates (+/+) at end-stage, immunolabelled with antibodies against SLP2, PHB2 and Vimentin. Scale bar = 10 µm. (C) Lumbar spinal cord sections from Chchd10S59L/+ mice (S59L/+) and control littermates (+/+) at end-stage. Immunolabelling was performed with antibodies against CHCHD10, PHB2 and Vimentin. Scale bar = 10 µm. (D) Lumbar spinal cord sections from Chchd10S59L/+ mice (S59L/+) and control littermates (+/+) at end-stage, immunolabelled with antibodies against CHCHD10, SLP2 and Vimentin. Scale bar = 10 µm. (E) Lumbar spinal cord sections from Chchd10S59L/+ mice (S59L/+) at end-stage. Left, TUNEL assay was combined with immunolabelling by using antibodies against SLP2 and Vimentin. Arrows indicate apoptotic (TUNEL+/DAPI+/SLP2+/Vimentin+) neurons. Asterix indicates a DAPI+/TUNEL/SLP2/Vimentin neuron. Right, Quantification of SLP2+/Vimentin+ neurons (Supplementary Fig. 6C) displaying TUNEL-positive signal. n = 3 independent experiments from three Chchd10S59L/+ mice and three control littermates. A total of 96–103 SLP2+/Vimentin+ MNs from S59L/+ mice or control littermates was analysed. Scale bar = 20 µm.

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