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lüll Cultured skin fibroblasts in storage disorders An analysis of ultrastructural features Kamensky E; Philippart M; Cancilla P; Frommes SPAm J Pathol 1973[Oct]; 73 (1): 59-80Electron microscopic studies were performed on cultured fibroblasts from patients with metachromatic leukodystrophy, Fabry's, Gaucher's, Niemann-Pick's (Type A and C), Sanfilippo's (Type A and B) disease, chondroitin-4-sulfate mucopolysaccharidosis, lipofuscinosis (Spielmeyer-Vogt's disease) and ceroid-lipofuscinosis (Batten's disease with curvilinear bodies). Specific cytoplasmic inclusions with a limiting membrane were identified in Fabry's disease, Niemann-Pick syndrome, chondroitin-4-sulfate mucopolysaccharidosis and Sanfilippo's Type B disease. In Fabry's disease, the lipid inclusions tended to form stacks of parallel and concentric membranes. In Niemann-Pick syndrome, the lipid inclusions were made of wavy, loosely packed membranes. In chondroitin-4-sulfate mucopolysaccharidosis and Sanfilippo B, the lysosomes were enlarged and contained a reticular matrix with little electron-dense material. No specific ultrastructural changes were observed in Gaucher's, Sanfilippo's (Type A) disease, metachromatic leukodystrophy (sulfatidosis) and Batten's disease.|*Cells, Cultured[MESH]|*Fibroblasts/cytology[MESH]|Biopsy[MESH]|Brain/pathology[MESH]|Carbohydrate Metabolism, Inborn Errors/*pathology[MESH]|Cell Nucleolus[MESH]|Cell Nucleus[MESH]|Cytoplasm[MESH]|Endoplasmic Reticulum[MESH]|Fabry Disease/pathology[MESH]|Female[MESH]|Gaucher Disease/pathology[MESH]|Golgi Apparatus[MESH]|Humans[MESH]|Leukodystrophy, Metachromatic/pathology[MESH]|Lipid Metabolism, Inborn Errors/*pathology[MESH]|Liver/pathology[MESH]|Male[MESH]|Microscopy, Electron[MESH]|Mitochondria[MESH]|Mucopolysaccharidoses/pathology[MESH]|Niemann-Pick Diseases/pathology[MESH]|Ribosomes[MESH]|Skin/*pathology[MESH] |