Using two previously described cohorts of human AVM subjects and their controls (toxic AVM cohort [11] and IBM cohort [15]), we found that Keap1 is diffusely distributed in the sarcoplasm of normal muscle fibers (Fig. 1 and Additional file 2: Figure S1) but is sequestered into the SQSTM1-positive sarcoplasmic protein aggregates in the AVM muscle (Figs. 1, 3 and 5); in fact, Keap1-positive sarcoplasmic puncta/inclusions can be used as a diagnostic marker for both toxic AVMs (Fig. 2) and IBM (Fig. 4). Here, KEAP1 is linked to inclusion body myositis.