Hexosaminidase is an enzyme that hydrolyzes terminal N-acetyl-D-hexosamine residues from N-acetyl-β-D-hexosaminides. The enzyme, localized to cellular lysosomes, is a dimer formed from ei ther an α or ß subunit which produces three possible dimer combinations. The αß combination (isozy...
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 fi- hexosaminidase-A. In Animal Models of Inherited Metabolic Diseases. R. J. Desnick, D. F. Patterson, and D. G. Scarpelli, editors. Alan R. Liss, Inc., NY. 213-220....
When offered as micelles, GD1a-GalNAc was almost not hydrolyzed by Hex A or Hex B, while after incorporation of the ganglioside into the outer leaflet of liposomes, the terminal GalNAc residue was rapidly split off by Hex a. In striking contrast to ganglioside GM2, the major glycolipid ...
产品名 Beta-Hexosaminidase B (HEXB) 货号 EHC27201 描述 Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosami...
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....
Agric. Biol. Chem. 55(11) , 2817-23, (1991) beta-N-Acetylhexosaminidase was produced by Trichoderma harzianum cultivated with chitin as the growth substrate. The enzyme was purified 13.2-fold to homogeneity by ultrafiltration and sequential chr... 详情 英文...
We have isolated a cDNA clone containing sequences complementary to mRNA encoding the alpha-chain of the lysosomal enzyme beta-hexosaminidase. RNA from a human lung fibroblast strain, IMR90, was enriched for beta-hexosaminidase messenger by polysome immunoselection with antiserum against beta-hexosamini...
306,307 The N-(3-thiopropanoyl) derivative 145 in complex with recombinant enzyme (PDB 2XPK) showed the acyl residue in a “selectivity pocket,” which distinguishes the enzyme from the human lysosomal hexosaminidases, which feature more shallow pockets that cannot accommodate the extended acyl ...
sphingomyelinase gene, alpha-L-iduronudase gene, huntingtin gene, neurofibromin 1 gene, MLH1 gene, MSH2 gene, MSH6 gene, PMS2 gene, cystic fibrosis transmembrane conductance regulator gene, hexosaminidase A gene dystrophin gene, FMR1 gene, phenylalanine hydroxylase gene and low-density lipoprotein...