Beta glucuronidase Recombinant Rabbit Monoclonal Antibody [JE54-34] (ET7110-66)

UNIPROT #

P08236

PROTEIN NAME

Beta glucuronidase

SYNONYMS

Ac2-223 antibody;asd antibody;Beta G1 antibody;Beta glucuronidase antibody;Beta-G1 antibody;Beta-glucuronidase antibody;BG antibody;BGLR antibody;BGLR_HUMAN antibody;FLJ39445 antibody;Glucuronidase beta antibody;Gur antibody;Gus antibody;Gus-r antibody;Gus-s antibody;Gus-t antibody;Gus-u antibody;GUSB antibody;Gut antibody;MPS7 antibody

SEQUENCE SIMILARITIES

Belongs to the glycosyl hydrolase 2 family.

POST-TRANSLATIONAL MODIFICATION

N-linked glycosylated with 3 to 4 oligosaccharide chains.

SUBCELLULAR LOCATION

Lysosome.

FUNCTION

Beta-glucuronidases are members of the glycosidase family of enzymes that catalyze breakdown of complex carbohydrates.Human β-glucuronidase is a type of glucuronidase (a member of glycosidase Family 2) that catalyzes hydrolysis of β-D-glucuronic acid residues from the non-reducing end of mucopolysaccharides (also referred to as glycosaminoglycans) such as heparan sulfate. Human β-glucuronidase is located in the lysosome. n the gut, brush border β-glucuronidase converts conjugated bilirubin to the unconjugated form for reabsorption. Beta-glucuronidase is also present in breast milk, which contributes to neonatal jaundice. The protein is encoded by the GUSB gene in humans and by the uidA gene in bacteria. Deficiencies in β-glucuronidase result in the autosomal recessive inherited metabolic disease known as Sly syndrome or Mucopolysaccharidosis VII. A deficiency in this enzyme results in the build-up of non-hydrolyzed mucopolysaccharides in the patient. This disease can be extremely debilitating for the patient or can result in hydrops fetalis prior to birth.