2.3.1.78	acetyl-CoA + heparan sulfate alpha-D-glucosaminide	-	
2.3.1.78	acetyl-CoA + heparan sulfate alpha-D-glucosaminide	initial step in heparan sulfate degradation	
2.3.1.78	acetyl-CoA + heparan sulfate alpha-D-glucosaminide	mucopolysaccharidosis IIIC (MPS IIIC, or Sanfilippo C syndrome) is a lysosomal storage disorder caused by the inherited deficiency of the lysosomal membrane enzyme acetyl-coenzyme A:alpha-glucosaminide N-acetyltransferase, which leads to impaired degradation of heparan sulfate. Structural protein that transports the activated acetyl residues across the cell membrane	
2.3.1.78	additional information	enzyme deficiency leads to Sanfilippo syndrome type C, i.e. mucopolysaccharidosis III C	
2.3.1.78	additional information	enzyme is acetylated at the cytoplasmic side of the lysosome and the acetyl group is then transferred to the inside where it is used to acetylate heparan sulfate	
2.3.1.78	additional information	enzyme deficiency causes mucopolysaccharidosis type IIIC