Fig. 1
From: Activation of the Keap1/Nrf2 stress response pathway in autophagic vacuolar myopathies
Keap1 immunohistochemistry labels coarse sarcoplasmic puncta in toxic AVM muscle. a-d. Faint diffuse sarcoplasmic staining is seen in muscle samples from a normal control subject (#10; a-b) and a drug-treated control subject (#17; c-d). e-h. Frequent fibers with the Keap1-positive coarse sarcoplasmic puncta are observed in muscle specimens from subjects in the toxic AVM group. The Keap1-positive puncta were often seen in the center of the fiber (aligned with the longitudinal fiber axis; e) or adjacent to/within sarcoplasmic vacuoles (arrowheads, f); in other cases, the puncta were seen throughout the fiber sarcoplasm (g-h). (e-f, HCQ-treated subject #25; g-h, colchicine-treated subject #22). Scale bars, 50 μM