Immunoblots and relative mRNA levels of of endoplasmic reticulum molecular chaperones in normal control and GNE myopathy muscle biopsies.

<p>A: Immunoblots of muscle homogenates of two normal control and five GNE myopathy muscle biopsies demonstrate in GNE myopathy a much stronger expression of GRP94, GRP78, calnexin and calreticulin except for ERp72 as compared to control muscle biopsies. C = control tissue, n = 2 (Samples are from control-1 and -2); GNE myopathy, n = 5 (Samples are from HIBM2-1, -2, -3, -4, and -5) B: Densitometric analysis of the blots in A performed using Quantity One. Protein loading was evaluated by the actin band. Data are indicated as mean±SD. Significance was determined by one-tailed unpaired t-test. The level of significance was set at P<0.05.C: Relative mRNA levels of molecular chaperones in normal and GNE myopathy patient biopsies. A total of four samples for each group were used, and each sample was run in triplicate for real-time PCR. C = control tissue, n = 4 (Samples are from control-1, -3, -4 and -5); GNE myopathy, n = 4 (Samples are from HIBM-5, -6, -7 and -8). Relative mRNA levels of four molecular chaperones were significantly increased in GNE myopathy muscle biopsies, GRP94 to112.3%, GRP78 to78.1%, calnexin to165.5%, and calreticulin to 189.6%. Data are shown as means ± SD. The level of significance was set at P<0.05. The densitometry graphs are representative of only the one chosen in the corresponding blots.