β-glucuronidase

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Β-glucuronidase
Β-glucuronidase
Ribbon / surface model of the dimer with nonaglycoside according to PDB  1BHG
Properties of human protein
Mass / length primary structure 629 amino acids
Secondary to quaternary structure Homotetramer
Isoforms Long, short
Identifier
Gene name GUSB
External IDs
Enzyme classification
EC, category 3.2.1.31 glycosidase
Response type hydrolysis
Substrate β-D-glucuronoside + H 2 O
Products D-glucuronate + alcohol
Occurrence
Homology family Beta-glucuronidase
Parent taxon Creature
Exceptions plants

β-Glucuronidase (GUSB) is the name for enzymes that split β-glucuronides, i.e. glycosides that are derived from glucuronic acid . This reaction step is indispensable for animals and some bacteria in the breakdown of the substances heme , chondroitin sulfate and keratan sulfate . The enzyme is found in the lysosomes of the liver , spleen and tonsils . Defects in the GUSB gene in humans can cause type 7 mucopolysaccharidosis and play a role in hydrops fetalis .

pathology

In the event of a functional deficiency in β-glucuronidase, there is a hereditary disease , a beta-glucuronidase deficiency, which is known as Sly syndrome and belongs to the mucopolysaccharidoses (type VII).

use

It finds applications in biotechnology as a reporter gene . Their activity can be proven using various methods:

See also

Individual evidence

  1. UniProt entry
  2. Entry on β-glucuronidase. In: Römpp Online . Georg Thieme Verlag, accessed on December 29, 2014.
  3. Mucopolysaccharidosis type 7. In: Orphanet (database for rare diseases).

Web links