Fig. 3
From: ‘Dusty core disease’ (DuCD): expanding morphological spectrum of RYR1 recessive myopathies
Ultrastructural findings of DuCD. Large irregular areas of sarcomeric disorganization with longer longitudinal axis and shorter transversal axis (a) containing abundant electrodense longitudinally-smeared material (arrows) and thickened short Z-line darker fragments (arrowheads), in longitudinal (a and b) and trasversal (c) sections. Smaller areas of sarcomeric disorganization (few sarcomeres) were alsodetected (d). Areas of disorganization occupying the entire muscle fibre in width with centralized/internalised nuclei (e). Several triads duplication or multiplication were detected inside dusty cores (black arrows). (a, d and e: p10, 34 years; b: p17, 34 years; c and f: p7, 76 year)