Supplemental Figure S8.

Pial vessel pathology in the Asah1^P361R/P361R mice. Representative electron micrograph featuring vascular cells in the ON of 8- to 9-week–old Asah1^+/+ and Asah1^P361R/P361R mice. A: Normal structured pial vessel in an Asah1^+/+ mouse. B and C: Pial vessel in an Asah1^P361R/P361R mouse that is thickened by accumulation of vacuolated cells with macrophage and fibroblast morphology. B and C: Storage pathology is also present in the endothelial cells that line the optic vein. White dagger, endothelial cell nucleus; black dagger, macrophage nucleus; section sign, eosinophil granulocyte nucleus; white asterisk, smooth muscle cell nucleus; black asterisk, astrocyte nucleus; black arrows, zebra body–like storage bodies; black arrowheads, eosinophil granulocytes; white arrowheads, partly cleared storage vacuoles with curvilinear tubular (Farber) bodies. Scale bar = 2 μm.