Extended Data Fig. 6: Sarcoplasmic RBM20-R636S rosary beads compared with TDP-43 myo-granules in RBM20-DCM myocardium.
From: Dysregulated ribonucleoprotein granules promote cardiomyopathy in RBM20 gene-edited pigs
a, Expanding on the results shown in (Fig. 3c), RBM20 IF-IHC confocal microscopy of human RBM20-DCM myocardium highlighting rosary beads-on-a-string staining pattern when confocal plane is focused on sarcomeres in longitudinal orientation. Repeated three times, this experiment produced identical results. Scale bar, μm. b, Double IF-IHC confocal microscopy for TARDBP (TDP-43), the driver of myo-granules in skeletal muscle, and RBM20 in HMZ pig myocardium highlighting distinctive (non-coincident) linear staining patterns of these two RNA-binding proteins. Repeated three times, this experiment produced identical results. Scale bar, μm. c, Corroborating the results observed in (b) above, GFP-RBM20-R636S transfected human iPSC-CMs highlighting distinctive fibrillary TARDBP by ICC and droplet-like RBM20-R636S RNP granules by confocal microscopy. Scale bar, μm.
