Fig. 3
Histochemical features of myoglobinopathy. a Anterior tibialis muscle biopsy from individual F3, III: 15, 10 years prior to the onset of symptoms, stained with hematoxylin and eosin, showing several rounded brown inclusions (arrows) (sarcoplasmic bodies) in the majority of myofibers and very small vacuoles in some myofibers (arrowhead in a). b, c Biceps brachii from individual F1, II: 7, 15 years after disease onset. b Note the presence of collections of sarcoplasmic bodies within the rimmed vacuoles. d, e Sarcoplasmic bodies appear red on modified Gomori trichrome stain. e In muscle biopsies with more advanced pathological lesions, large numbers of rimmed vacuoles are observed. f No major architectural changes are seen on NADH reaction, apart from lack of oxidative activity at the site of vacuoles. g Fast myosin immunohistochemistry demonstrate the presence of sarcoplasmic bodies in both type 1 (slow) and 2 (fast) myofibers. h Myofiber regions containing vacuoles display strong phosphatase activity, and LAMP1 i immunoreactivity. Scale bar in a, e, f, g, and h = 50 µm; scale bar in b, c, d, and i = 20 µm
