Loss of EHBP1L1 causes abnormal central positioning of nuclei and mitochondria in skeletal muscle cells. (A-B) Representative images of HE staining (A) and immunofluorescence staining for the mitochondrial marker COXIV (B) in hindlimb muscles from E18.5 embryos are shown. The numbers of skeletal muscle cells with centralized nuclei (yellow arrows in panel A) and centrally accumulated mitochondria (white arrows in panel B) were determined (6 visual fields for each genotype in panel A; 4 visual fields for control mice and 5 visual fields for Ehbp1l1^−/− mice in panel B). Bars: 20 μm for panel A and 10 μm for panel B. ∗∗P < .01, ∗∗∗∗P < .0001 (Fisher exact test). (C) A schematic diagram illustrating the function of EHBP1L1 in terminal erythropoiesis is shown.