Characterization of brain dystrophins absence and impact in dystrophin-deficient Dmdmdx rat model
Fig 7
Lack of Dp427 dystrophin isoform in Dmdmdx rats brain and no modification in distribution of Dp140 and Dp71 isoforms.
Males WT controls (n = 3) and Dmdmdx rats (n = 3) were sacrificed and slides from brain areas (at the levels of the frontal pole, the optic chiasm, the oculomotor nerve, the midbrain and the occipital pole) were processed for immunolabelling of Dp71, Dp140 and Dp427 dystrophin isoforms using respectively monoclonal antibodies NCL-DYS2, MANEX5556 and NCL-DYSB. Isoforms Dp71 and Dp140 were detected with similar intensities in WT littermate controls and Dmdmdx rats and were respectively localized in endothelial cells of blood capillaries and in smooth muscle layer of blood vessels throughout brain parenchyma. Isoform Dp427 was mostly detected in the cytoplasm of some large neurons of the hippocampus in WT controls but was absent in Dmdmdx littermate rats.