14800-DvRappard

97 Donor macrophages and remyelination in metachromatic leukodystrophy 6 Figure 4. Transplantation prevents loss of white matter oligodendrocytes. Stain with Klüver-PAS of whole mount coronal brain slices of an untreated (A, patient 3) and a transplanted patient (B, patient 1) shows a variable degree of myelin loss in the periventricular and deep white matter, with relative sparing of the subcortical white matter and U-fibers. (C, patient 4) Klüver-PAS stain of an untreated patient shows the centrifugal progression of the demyelinating process, with the periventricular white matter (below the dotted line) containing less myelin than the subcortical white matter (between dotted and solid line). (D) Quantification of immunoreactivity against the myelin protein proteolipid protein (PLP) confirms that myelin loss is more marked in the deep white matter of untreated compared to transplanted patients, whereas myelin amounts in the subcortical frontal and parietal regions are comparable. (E-G) Stain against the oligodendrocyte lineage-specific marker olig2 shows marked loss of oligodendrocytes in untransplanted patients (E, patient 6), but preservation of cells in transplanted patients (F, patient 2). In treated patients (F), cells with more or less intense immunoreactivity are appreciable, corresponding to oligodendrocyte progenitors and mature cells. Quantification (G) confirms that oligodendrocyte numbers are much higher in the white matter of treated compared to untreated patients.