3.10.1.1	heparan sulfate + H2O	-	
3.10.1.1	heparan sulfate + H2O	degradation of heparan sulfate	
3.10.1.1	heparin + H2O	-	
3.10.1.1	heparin + H2O	degradation products of	
3.10.1.1	additional information	congenital deficiency of sulfamidase leads to mucopolysaccharidosis type IIIA or Sanfilippo syndrome, a lysosomal storage disorder, with consequent accumulation of partially degraded heparan sulfate in lysosomes and the central nervous system as the predominant site of tissue damage, overview	
3.10.1.1	additional information	enzyme defiency leads to defective lysosomal degradation of the glycosaminoglycan heparan sulfate, mutations of the enzyme are responsible for mucopolysaccharidosis type IIIA, i.e. Sanfilippo A syndrome, onset and progression of the disease, overview	
3.10.1.1	additional information	reduced activity of sulfamidase results in intracellular accumulation of heparan sulfate, with the brain the primary site of pathology, e.g. in mucopolysaccharidosis type IIIA, or Sanfilippo syndrome, an inherited neurodegenerative lysosomal storage disorder, progressive loss of learned skills, sleep disturbance and behavioural problems occur. A potential therapy method is the repeated injection of the enzyme into cerebrospinal fluid via cisterna magna leading to a reduction of the number of lysosomal storage inclusions in the brain with a significant decrease in the immunohistochemical staining of a lysosomal membrane marker, and to reduced numbers of activated isolectin-B4-positive microglia and GFAP-positive astrocytes in some brain regions, phenotype, mouse model, overview	
3.10.1.1	additional information	repeated human enzyme injection into murine cerebrospinal fluid via cisterna magna leads to a reduction of heparan sulfate-derived monosulfated disaccharide in the brain and spinal cord, reduced lysosomal vesicle formation in various cell types, reduced axonal spheroids, and improved behaviour of treated mice, mouse model, overview