Mucopolysaccharidosis 3 A (Sanfilippo A disease): deficiency of a heparin sulfamidase in skin fibroblasts and leucocytes.

Acetylesterase/metabolism Animals Aorta/metabolism Carbohydrate Metabolism, Inborn Errors/enzymology Cattle Fibroblasts/enzymology Heparin Humans Hydrogen-Ion Concentration Hydrolases In Vitro Techniques Intellectual Disability/enzymology Leukocytes/enzymology Mucopolysaccharidoses/metabolism Skin/enzymology Sulfatases/metabolism Sulfates/metabolism Sulfur Radioisotopes

Sulfates Sulfur Radioisotopes Heparin Hydrolases Acetylesterase Sulfatases N-sulfoglucosamine sulfohydrolase