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Figure 8 | BMC Neurology

Figure 8

From: Nuclear entrapment and extracellular depletion of PCOLCE is associated with muscle degeneration in oculopharyngeal muscular dystrophy

Figure 8

Nuclear localisation of PCOLCE is enriched in OPMD compared to other muscular dystrophies. A. Sub-cellular localisation of PCOLCE in DMD, FSHD and DM1 patient muscle. Cryosections were stained for PCOLCE and Dysferlin. Dysferlin shows a sarcolemmal localisation. In DMD and FSHD biopsies there is no clear reduction of PCOLCE at the ECM. In DM1 areas with reduced PCOLCE at the ECM are indicate with arrows. Scale bar equals 10 μm. B. Accumulation of PCOLCE in myonuclei was quantified using overlap in intensity distribution between DAPI (blue) and PCOLCE (red). Plots show two nuclei without enrichment with nuclear PCOLCE (i) or with PCOLCE enrichment (ii). Histogram (iii) shows the quantification of 100 nuclei from FSHD, DMD, DM1 and OPMD muscle biopsies.

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BMC Neurology

ISSN: 1471-2377

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