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LysosomalHexosaminidaseCatalyzeLinkagesAmino acidActivityReplacementPotentialFamilyPatientsDevelopmentBroadly distributedSubstrateMajorTypes
Lysosomal3
  • Most lysosomal enzyme deficiencies are catastrophic illnesses with no generally available treatments. (mcmaster.ca)
  • Elevated secondary lysosomal enzymes beta-hexosaminidase and alpha-galactosidase were also reduced. (mcmaster.ca)
  • These enzymes play a vital role in a wide variety of biological events, such as digestion of nutritional carbohydrates, lysosomal catabolism of glycoconjugates, and posttranslational modifications of glycoproteins. (bvsalud.org)
Hexosaminidase3
  • The hexA and hexB genes encode the alpha- and beta-subunits of the two major beta-N-acetylhexosaminidase isoenzymes, N-acetyl-beta-D-hexosaminidase A (HexA) and beta-N-acetylhexosaminidase B (HexB). (unl.edu)
  • Pediatric reference data on activity of urinary N-acetyl-beta-D-hexosaminidase and its isoenzymes. (edu.pl)
  • Reference data on neonatal serum N-acetyl-beta-hexosaminidase activity. (edu.pl)
Catalyze2
  • Beta-N-acetylhexosaminidases of glycosyl hydrolase family 20 (GH20) catalyze the removal of beta-1,4-linked N-acetyl-D-hexosamine residues from the non-reducing ends of N-acetyl-beta-D-hexosaminides including N-acetylglucosides and N-acetylgalactosides. (unl.edu)
  • Glycosidases are ubiquitous enzymes that catalyze the hydrolysis of glycosidic linkages in oligosaccharides and glycoconjugates. (bvsalud.org)
Linkages2
  • A functionally uncharacterized subgroup of the Glycosyl hydrolase family 20 (GH20) catalytic domain found in proteins similar to the chitobiase of Serratia marcescens, a beta-N-1,4-acetylhexosaminidase that hydrolyzes the beta-1,4-glycosidic linkages in oligomers derived from chitin. (unl.edu)
  • The chitobiase of Serratia marcescens is a beta-N-1,4-acetylhexosaminidase with a glycosyl hydrolase family 20 (GH20) domain that hydrolyzes the beta-1,4-glycosidic linkages in oligomers derived from chitin. (unl.edu)
Amino acid1
  • The recent crystal structure of a family 3 enzyme from ' E. coli' was used to predict logical amino acid replacements in order to identify the putative catalytic acid and substrate-binding domains in sMltB-His 6 and SltBl-His6. (uoguelph.ca)
Activity3
  • However, ' O'-acetylation of the C-6 of MurNAc appeared to inhibit activity as predicted by the proposed mechanism of these enzymes. (uoguelph.ca)
  • After 24 hr, beta-glucuronidase activity was detected in the plasma, reaching 66% of physiological levels by 2 weeks postimplantation. (mcmaster.ca)
  • Significant beta-glucuronidase activity was detected in liver and spleen for the duration of the 8-week experiment. (mcmaster.ca)
Replacement1
  • A "universal" cell line engineered to secrete the missing enzyme is implanted in all recipients requiring the same enzyme replacement. (mcmaster.ca)
Potential1
  • However, implanted mutant MPS VII mice developed antibodies against the murine beta-glucuronidase, demonstrating a potential obstacle in patients with a null mutation who react against the replaced enzyme as a foreign antigen. (mcmaster.ca)
Family1
  • Both the alpha and the beta catalytic subunits have a TIM-barrel fold and belong to the glycosyl hydrolase family 20 (GH20). (unl.edu)
Patients1
  • Beutler also developed an early interest in iron metabolism, prompted by his clinical observation of the rapid symptomatic improvement of iron deficient patients treated with iron: an improvement that preceded any major hematologic change, and showed that numerous enzymes were sensitive to iron deficiency. (wikipedia.org)
Development1
  • Owing to the physiological and pathological significance of glycosidases, the development of small molecules that target these enzymes is an active area in glycoscience and medicinal chemistry. (bvsalud.org)
Broadly distributed2
  • The enzymes involved, the hyaluronidases, are broadly distributed. (gr.jp)
  • These enzymes are broadly distributed in microorganisms, plants and animals, and play roles in various key physiological and pathological processes. (unl.edu)
Substrate2
  • The concentration-dependent effect of these variants on hydrolysis of the synthetic substrate 4-methylumbelliferyl-2-acetamido-2-deoxy-6-sulfo-beta-D-glucopyranoside (MUGS) indicated a weakened association with the enzyme's alpha subunit. (nih.gov)
  • The GH20 hexosaminidases are thought to act via a catalytic mechanism in which the catalytic nucleophile is not provided by solvent or the enzyme, but by the substrate itself. (unl.edu)
Major2
  • Beutler also developed an early interest in iron metabolism, prompted by his clinical observation of the rapid symptomatic improvement of iron deficient patients treated with iron: an improvement that preceded any major hematologic change, and showed that numerous enzymes were sensitive to iron deficiency. (wikipedia.org)
  • 1) Mammalian-type hyaluronidases (EC 3.2.1.35) are endo-beta-N-acetylhexosaminidases with tetrasaccharides and hexasaccharides as the major endproducts. (gr.jp)
Types1
  • The different types of hyaluronidases were originally classified into three distinct classes by him, a scheme based on biochemical analysis of the enzymes and their reaction products. (gr.jp)