GUSB
GUSB (glucuronidase beta, also known as beta-glucuronidase) is a lysosomal hydrolase that degrades glycosaminoglycans. It plays an important role in the breakdown of dermatan and keratan sulfates, and mutations in the GUSB gene cause mucopolysaccharidosis type VII. Browse Assay Genie's current range of GUSB research reagents below, or read on for the biology behind the target.
GUSB is a hydrolase that degrades glycosaminoglycans. UniProt annotates it as playing an important role in the degradation of dermatan and keratan sulfates, and the NCBI Gene summary adds heparan sulfate and chondroitin-4,6-sulfate to its substrates. The enzyme is classified as a beta-glucuronidase (EC 3.2.1.31). It acts within the lysosome, where glycosaminoglycan breakdown takes place.
The human GUSB gene encodes the beta-glucuronidase protein, which belongs to the glycosyl hydrolase 2 family and, in HGNC, to the glycoside hydrolase family gene group. The mature enzyme forms a homotetramer and is localised to the lysosome. Alternative splicing of the gene produces multiple transcript variants. The human genome also contains many pseudogenes of this locus, which is a useful point to bear in mind when designing nucleic acid assays. Mouse and rat both have an orthologous gene, Gusb, so the enzyme can be studied in common rodent models.
Mutations in GUSB result in mucopolysaccharidosis type VII, a disorder of glycosaminoglycan degradation. This link makes GUSB a reference point in lysosomal storage research. The gene was previously named glucuronidase, beta, and the current HGNC name is glucuronidase beta. Researchers working with GUSB antibody, GUSB ELISA kit or GUSB protein reagents will find that the protein's lysosomal location and tetrameric structure are important considerations for experimental design.
Mutations in GUSB cause mucopolysaccharidosis type VII, making the enzyme central to studies of impaired glycosaminoglycan degradation.
GUSB degrades dermatan sulfate, keratan sulfate, heparan sulfate and chondroitin-4,6-sulfate, so it is relevant to studies of glycosaminoglycan turnover.
As a lysosomal homotetrameric hydrolase, GUSB is used to examine lysosomal enzyme function and localisation.
GUSB belongs to the glycosyl hydrolase 2 family and is a beta-glucuronidase, offering a model for studying this enzyme class.
GUSB is a lysosomal hydrolase that degrades glycosaminoglycans. UniProt describes an important role in degrading dermatan and keratan sulfates, and NCBI Gene lists heparan sulfate and chondroitin-4,6-sulfate as further substrates. The active enzyme is a homotetramer.
The protein is called beta-glucuronidase, and HGNC gives the gene name as glucuronidase beta, previously glucuronidase, beta. No other aliases are listed. NCBI Gene notes that alternative splicing produces multiple transcript variants of the gene.
Mutations in GUSB result in mucopolysaccharidosis type VII. The gene has orthologs named Gusb in both mouse and rat. Check the datasheet of any product for its validated reactivity.
Other lysosomal hydrolases and glycosaminoglycan-degrading enzymes studied alongside GUSB in lysosomal storage research.
Gene and protein annotations from HGNC, NCBI Gene and UniProt P08236; product specifications from Assay Genie datasheets.