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. 2016 Oct 13;99(5):1086–1105. doi: 10.1016/j.ajhg.2016.09.005

Figure 5.

Figure 5

PYROXD1 Shows Both Nuclear and Striated Immunolocalization in Human Muscle

(A) Control stretched human skeletal (quadriceps) muscle co-stained with α-PYROXD1 (red), myotilin or desmin (green), and DAPI (blue). PYROXD1 intensely labels peripheral myonuclei, as well as showing two different patterns of striated labeling. Right: In this fiber, PYROXD1 brightly labels consecutive myonuclei and shows cytoplasmic striated labeling that aligns with desmin (Z-band region) and interdigitates between Z-bands (M-line region). Left: PYROXD1 does not brightly label myonuclei (asterisks show the position of DAPI-labeled nuclei) and shows a broader banding of striated labeling spanning the breadth of the I-band.

(B) Control and AII-1 tricep cross-sectioned human skeletal muscle co-stained with α-PYROXD1 (red), α-desmin (green), and DAPI (blue). The triceps muscle from AII-1 bears large inclusions that positively label for desmin, split fibers, and multiple internalized nuclei that brightly label for PYROXD1. Coverslips were imaged on a Leica SP5 confocal and single Z-planes are presented.