HGSNAT oligomer acetylates Heparan sulfate chain(3)
Ac-CoA + GlcN-IdoA2S-GlcNS3S- group → CoA-SH + GlcNAc-IdoA2S-GlcNS3S- group + H+
- Reactome
- R-HSA-1678660
- Compartment
- lysosomal membrane · cytosol · lysosomal lumen
- Rhea
- RHEA:15126
Inputs
- Ac-CoACHEBI:57288, C23H34N7O17P3S, cytosol
- GlcN-IdoA2S-GlcNS3S- groupCHEBI:63806, C41H67N2O43S3, lysosomal lumen
Outputs
- CoA-SHCHEBI:57287, C21H32N7O16P3S, cytosol
- GlcNAc-IdoA2S-GlcNS3S- groupCHEBI:63807, C43H69N2O44S3, lysosomal lumen
- H+CHEBI:15378, H, lysosomal lumen
Catalysed by
- heparan-alpha-glucosaminide N-acetyltransferase activity of HGSNAT oligomer [lysosomal membrane]EC 2.3.1.78
Literature
- Analysis of the biogenesis of heparan sulfate acetyl-CoA:alpha-glucosaminide N-acetyltransferase provides insights into the mechanism underlying its complete deficiency in mucopolysaccharidosis IIIC J Biol Chem, 2010
- Identification of the gene encoding the enzyme deficient in mucopolysaccharidosis IIIC (Sanfilippo disease type C) Am J Hum Genet, 2006
- Mutations in TMEM76* cause mucopolysaccharidosis IIIC (Sanfilippo C syndrome) Am J Hum Genet, 2006