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Fig. 8 | Acta Neuropathologica Communications

Fig. 8

From: New insights into the protein aggregation pathology in myotilinopathy by combined proteomic and immunolocalization analyses

Fig. 8

Immunolocalization of proteins involved in unfolded protein response (UPR), oxidative stress response and transcription regulation. Serial skeletal muscle cryosections from a myotilinopathy patient (ID1 in Table 1) were double-stained with antibodies recognizing GRP78/BiP, thrombospondin 4 (both involved in UPR), MGST3 (related to oxidative stress response), and YBX3 (transcription regulation) and either filamin C or myotilin. Nuclei were visualized by DAPI staining. Results of semiquantitative evaluation of signal intensities in the different abnormal areas A1–A3 are specified in Table 3. Scale bar = 50 μm

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