GUSB tetramer hydrolyses CS/HS precursor
CS/HS precursor + H2O → GlcA + linker chain(2)
- Reactome
- R-HSA-1678854
- Compartment
- lysosomal lumen
- Rhea
- RHEA:17634
Inputs
- CS/HS precursorCHEBI:747385, C23H37O21, lysosomal lumen
- H2OCHEBI:15377, H2O, lysosomal lumen
Outputs
- GlcACHEBI:4178, C6H10O7, lysosomal lumen
- linker chain(2)CHEBI:154964, C17H30O15, lysosomal lumen
Catalysed by
- beta-glucuronidase activity of GUSB tetramer [lysosomal lumen]EC 3.2.1.31
Literature
- Cloning, sequencing, and expression of cDNA for human beta-glucuronidase Proc Natl Acad Sci U S A, 1987
- A novel inhibitor of beta-glucuronidase: L-aspartic acid Pediatr Res, 2001
- Mutations and polymorphisms in GUSB gene in mucopolysaccharidosis VII (Sly Syndrome) Hum Mutat, 2009