Autophagic abnormalities and autofluorescent inclusions in adult-onset patients. a-c: Muscle fibers were stained for lysosomal marker LAMP2 (green) and autophagosomal marker LC3 (red). (a) LOPD fiber (pt. D16) shows mild lysosomal enlargement and clusters of autophagosomes. (b) Autophagic buildup and autofluorescent inclusions represent a major pathology in this fiber (pt. D7). (c) Autofluorescent inclusions are seen within the LAMP2-positive structures (pt. D7). Bar in a-c: 10 μm. (d) Both autophagic buildup and inclusions are missed by routine histology; the image shows epon-embedded PAS-stained section of muscle biopsy from pt. D15 (10x).