Immunofluorescence shows AβPP and p-tau aggregates and molecule chaperones in GNE myopathy muscle.

<p>Single-label immunofluorescence illustrates strongly immunoreactive aggregates of AβPP (a1) in GNE myopathy, nonspecific staining in muscle interstitial of normal control (a2), aggregates of p-tau (a3) in GNE myopathy and nonspecific staining in muscle interstitial of normal control (a4). Single-label immunofluorescence illustrates strongly immunoreactive aggregates of calnexin (b1) in GNE myopathy, nonspecific staining in muscle interstitial of normal control (b2), aggregates of calreticulin in GNE myopathy (b3), nonspecific staining in muscle interstitial of normal control (b4), aggregates of GRP94 in GNE myopathy (c1), nonspecific staining in muscle interstitial of normal control (c2) and aggregates of GRP78 in GNE myopathy (d1) and nonspecific staining in muscle interstitial of normal control (d2). No positive staining of ERp72 was found in cytoplasm of GNE myopathy muscle fibers (e1) and normal control muscle fiber (e2). Double-label immunofluorescence illustrates that in GNE myopathy muscle fibers the aggregates immunoreactive for GRP94 (c3) are also immunoreactive for AβPP (d3) (e3 merged). Aggregates immunoreactive for GRP78 (c4) are also immunoreactive for AβPP (d4) (e4 merged). b1, d1 and e1 were from sample HIBM-1; c3, d3, e3, c4, d4 and e4 were from sample HIBM-2; c1 and b3 were from sample HIBM-3; a1 and a3 were from sample HIBM-5; a2 and a4 were from control-1; b2, d2 and e2 were from control-2; c2 and b4 were from control-3.