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. 2015 Sep 11;24(23):6588–6602. doi: 10.1093/hmg/ddv363

Figure 6.

Figure 6.

Z-disc proteins accumulate in DNAJB6b-F93L transgenic mouse muscle. (A–I) Dual immunofluorescence staining of 4-month old hDNAJB6b-WT or hDNAJB6b-F93L tibialis anterior muscle using an antibody to V5 that recognizes transgenic hDNAJB6b protein and an antibody to actinin (A–F) or desmin (G–I). Bar (A–F), 50 µm and Bar (G–I), 20 µm. (J) Immunoblot of tibialis anterior muscle lysates from three different 4-month old C57, DNAJBb-WT and DNAJB6b-F93L mice using antibodies to Z-disc proteins myotilin, α-actinin, desmin, syncolin and synemin. GAPDH is loading control. (K) qPCR analysis of desmin, myotilin and synemin expression in 4-month old hDNAJB6b-WT or hDNAJB6b-F93L mice. Data are presented as a fold change as compared with C57 controls. * represent P-value <0.01. (L) Tibialis anterior muscle lysates from 4-month old C57, DNAJB6b-WT and DNAJB6b-F93L mice were separated into a total, soluble and insoluble fraction. Fractions were then immunoblotted for V5 (to detect hDNAJB6b), desmin and α-actinin.