beta glucuronidase (GUSB, beta-G1, MPS7)

Function

• lysosomal hydrolase
• role in the degradation of dermatan & keratan sulfates N-linked glycosylated with 3 to 4 oligosaccharide chains

beta-D-glucuronoside + H2O <--> D-glucuronate + an alcohol

Structure

• homotetramer
• belongs to the glycosyl hydrolase 2 family

Compartment

lysosome

Alternative splicing

named isoforms=2

Expression

• leukocytes, other tissues ?
• present in urine

Pathology

• defects in GUSB are associated with mucopolysaccharidosis 7 & non-immune hydrops fetalis
• GUSB increases nonspecifically in many renal diseases
• tissue levels increase in many cancers, giving increased activity in body fluids, e.g.
  • pleural fluid {lung cancer}
  • gastric juice {stomach cancer}
  • vaginal fluid {cervical cancer}.

Genetics

Gene locus: 7q21.11

More general terms

• glycosidase (glycoside hydrolase)
• lysosomal hydrolase

More specific terms

• vestronidase alfa-vjbk (Mepsevii)

Additional terms

• mucopolysaccharidosis-7; Sly syndrome; MPS7

Database

• UniProt: http://www.uniprot.org/uniprot/P08236.html
• OMIM: https://mirror.omim.org/entry/253220
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=2990