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Phase II Reactions: Glucuronidation01:24

Phase II Reactions: Glucuronidation

Glucuronidation, a pivotal phase II biotransformation process, involves the coupling of glucuronic acid to a drug or xenobiotic. Given its widespread occurrence and critical role in drug metabolism, it's considered the most crucial phase II reaction. It enhances the water solubility of substances, aiding their expulsion from the body. The driving force behind these reactions is a group of enzymes known as UDP-glucuronosyltransferases (UGTs). UGTs facilitate the transfer of a glucuronic acid...
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In Vitro Enzyme Measurement to Test Pharmacological Chaperone Responsiveness in Fabry and Pompe Disease
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Published on: December 20, 2017

Human β-glucuronidase: structure, function, and application in enzyme replacement therapy.

Huma Naz1, Asimul Islam, Abdul Waheed

  • 11 Centre for Interdisciplinary Research in Basic Sciences , Jamia Millia Islamia, Jamia Nagar, New Delhi, India .

Rejuvenation Research
|June 20, 2013
PubMed
Summary
This summary is machine-generated.

Lysosomal storage diseases like mucopolysaccharidosis type VII result from deficient beta-glucuronidase (GUSB). This review covers GUSB

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Radiochemical Assessment of Glycogen Synthase Enzyme Activity in Animal Tissue
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Published on: October 24, 2025

Area of Science:

  • Biochemistry
  • Genetics
  • Molecular Biology

Background:

  • Lysosomal storage diseases (LSDs) arise from impaired breakdown of macromolecules within lysosomes.
  • Most lysosomal enzymes share common features like targeting motifs, despite structural variations.
  • Beta-glucuronidase (GUSB) is crucial for degrading glycosaminoglycans; its deficiency causes MPSVII, leading to brain lysosomal storage.

Purpose of the Study:

  • To consolidate current knowledge on the sequence, structure, function, and evolution of GUSB.
  • To review the lysosomal targeting mechanisms of GUSB.
  • To discuss enzyme replacement therapy for GUSB deficiency.

Main Methods:

  • Literature review of existing studies on GUSB.
  • Analysis of GUSB sequence, structure, and functional data.
  • Compilation of information on GUSB evolution and enzyme targeting.

Main Results:

  • GUSB is a well-characterized enzyme with known expression, sequence, and structural properties.
  • The review synthesizes information on GUSB's role in glycosaminoglycan degradation and MPSVII pathogenesis.
  • Lysosomal targeting pathways and evolutionary aspects of GUSB are detailed.

Conclusions:

  • A comprehensive understanding of GUSB is essential for addressing MPSVII.
  • Further research into GUSB targeting and enzyme replacement therapy holds therapeutic potential.
  • This review provides a foundational resource for GUSB-related research and therapeutic development.