Figure 3.

(A) Confocal immunofluorescence imaging of the patient muscle biopsy labeled with FHL1 antibody (red), lamin A/C antibody (green), and DAPI (blue). The maximum intensity projections of red and green channels (white) are shown on the left, the merged image of confocal projections are shown in the middle, and the 3D surface shaded reconstruction of an enlargement of the area defined by the white box is shown on the right. (B) Confocal immunofluorescence imaging of the patient muscle biopsy labeled with an anti-histones antibody (green) and DAPI (blue), together with 3D surface shaded reconstruction (merge). (C) Acridine-orange staining. The maximum intensity projections of the single channels (white) are shown on the left, the merged image of confocal projections are shown in the middle, and the 3D surface shaded reconstruction of an enlargement of the area defined by the white box is shown on the right. (D) Acridine-orange staining after DNase digestion on frozen sections of the patient’s muscle biopsy showing that the treatment completely removed nuclear and RBs associated DNA. The maximum intensity projections of the red and green channels (white) are shown on the left, the merged image of confocal projections are shown in the middle, and the 3D surface shaded reconstruction of the area defined by box is shown on the right. Scale bar, 10 μm.