Tay-Sachs disease (TSD) is a progressive neurodegenerative disorder that occurs due to a deficiency of a 尾 hexosaminidase A (HexA) enzyme, resulting in the accumulation of GM2 gangliosides. In this work, we analyzed the effect of umbilical cord blood cell transplantation (UCBCT) and...
Enzyme replacement in feline GM2 gangliosidosis: catabolic effects of human β-hexosaminidase A. Prog Clin Biol Res. 1982;94:213-220.Rattazzi M, Appel A, Baker H. Enzyme replacement in feline GM2 gangliosidosis: catabolic effects of human beta-hexosaminidase A. Prog Clin Biol Res 1982; 94:...
The product has an N-terminal amino acid sequence, specific activity, and size ( M r 62,000) similar to that of the enzyme present in cultured human fibroblasts. However, endo H sensitivity studies revealed that the oligosaccharide structures present on recombinant β-hexosaminidase B differ from...
Lending a helping hand, screening chemical libraries for compounds that enhance beta-hexosaminidase A activity in GM2 gangliosidosis cells. Enzyme enhancement therapy is an emerging therapeutic approach that has the potential to treat many genetic diseases. Candidate diseases are those associat... MB Tr...
Hexosaminidase B Beta (HEXb) BioAssay ELISA Kit (Mouse) is a sandwich enzyme immunoassay for in vitro quantitative measurement of HEXb in mouse serum, plasma, tissue homogenates and other biological fluids.Detection Range:0.156-10ng/mlSensitivity:0.062ng/mlStorage and Stability:Desiccation recommended....
Which one of the following enzyme deficiencies causes Gaucher disease? A. Beta-galactosidase B. Beta-glucocerebrosidase C. Beta-hexosaminidase A D. Beta-hexosaminidase B E. Beta-hexosaminidases A and B2. A couple in their 30s comes to a clinic for preconception counseling. The husband is Ashken...
The N-acetyl-β-D-hexosaminidase B of germinating L. luteus seeds catalysed the hydrolysis of both p-nitrophenyl-N-acetyl-β-D-glucosaminide and -galactosaminide substrates. The investigation of the pH dependence of the kinetic parameters Vmax and Vmax/Km demonstrated that 2 common ionizable grou...
The test principle applied in this kit is Sandwich enzyme immunoassay. The microtiter plate provided in this kit has been pre-coated with an antibody specific to Hexosaminidase B Beta (HEXb). Standards or samples are then added to the appropriate microtiter plate wells with a biotin-conjugated an...
Enzyme PrecursorsDNAProtein Processing, Post-TranslationalAmino Acid SequenceMolecular Sequence Databeta-N-AcetylhexosaminidasesHexosaminidase AThere are two major isozymes of human lysosomal beta-hexosaminidase (beta-N-acetylhexosaminidase, EC 3.2.1.52), hexosaminidase A, alpha(beta a beta b), and ...
The beta-linkage of the GlcNAc in the synthesized trisaccharide was confirmed by the action of the specific enzymes beta-hexosaminidase and beta-N-acetylglucosaminide beta 1-4-galactosyltransferase. Kinetic parameters were determined for UDP-GlcNAc, lactose, and N-acetyllactosamine. The enzyme ...