Fig. 1

Claudin-5, laminin and PDGFRβ expression in the frontal WM of non-neurological controls and leukodystrophy patients. Claudin-5 is expressed in between endothelial cells (UEA I) in all subjects. No peculiarities were seen in its expression in the leukodystrophies. Astrocytes are stained with GFAP and nuclei with DAPI. Laminin in the controls is, as expected, only expressed at the vasculature, where it stains the basal membrane. The expression of laminin is only altered in LARS2-related leukodystrophy, where astrocytes show cytoplasmic vesicular laminin immunoreactivity. Astrocytes are stained with GFAP, endothelial cells with UEA I and nuclei with DAPI. PDGFRβ in controls is only expressed in the vasculature, where it stains pericytes. The expression of PDGFRβ is not altered in leukodystrophies. Endothelial cells are stained with UEA I and nuclei with DAPI Scale bar = 25 µm