HGSNAT oligomer acetylates Heparan chain(1)
Ac-CoA + GlcN- group → CoA-SH + GlcNAc- group + H+
- Reactome
- R-HSA-2090085
- Compartment
- lysosomal membrane · cytosol · lysosomal lumen
- Rhea
- RHEA:15126
Inputs
- Ac-CoACHEBI:57288, C23H34N7O17P3S, cytosol
- GlcN- groupCHEBI:63811, C29H48NO24, lysosomal lumen
Outputs
- CoA-SHCHEBI:57287, C21H32N7O16P3S, cytosol
- GlcNAc- groupCHEBI:747401, C31H50NO26, 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