BACKGROUND GM2-gangliosidosis is a fatal neurodegenerative lysosomal storage disease (LSD) caused by deficiency of either β-hexosaminidase A (Hex-A) and β-hexosaminidase B (Hex-B) together, or the GM2 activator protein. Clinical signs can be variable and are not pathognomonic for the specific, causal deficiency. OBJECTIVES To characterize the phenotype and genotype of GM2-gangliosidosis disea...

The catabolism of Tay-Sachs ganglioside, N-acetylgalactosaminyl- (N-acetylneuraminosyl) -galactosylglucosylceramide, has been studied in lysosomal preparations from normal human brain and brain obtained at biopsy from Tay-Sachs patients. Utilizing Tay-Sachs ganglioside labeled with (14)C in the N-acetylgalactosaminyl portion or (3)H in the N-acetylneuraminosyl portion, the catabolism of Tay-Sac...

We investigated Candida albicans-induced mast cell degranulation in vitro and in vivo. Cell wall fraction but not culture supernatant and cell membrane fraction prepared from hyphally grown C. albicans induced β-hexosaminidase release in RBL-2H3 cells. Cell wall mannan and soluble β-glucan fractions also induced β-hexosaminidase release. Histological examination of mouse forestomach showed that...

Hexosaminidase, alpha-mannosidase, beta-galactosidase, beta-glucuronidase, and arylsulphatase A were measured inperitoneal and pleural effusions from patients with benign, malignant, and inflammatory disorders. Compared with the benign transudates, all enzyme activities were moderately elevated in malignant effusions and markedly elevated in inflammatory effusions. The assay of hexosaminidase a...

In the previous study, pickle-derived Lactiplantibacillus plantarum 22A-3 (LP22A3) suppressed ear edema in passive cutaneous anaphylaxis by its oral administration. Moreover, LP22A3 treatment directly to RBL-2H3 cells shows no effect on β-hexosaminidase release from but inhibited using Caco-2/RBL-2H3 co-culture system stimulated with apical side. this administration of decreased total IgE and o...

AMAcetyl-,-he,osanindases A andUwere purifled to homogeneityfromhuman placenta. 1n the imt'al step of purication, thfe enzymes were adsorbed on concanavalin ASepharose 4B and etuted from, the column with a-methyl D-mannoside. Subsequent purification steps includedc DEAE-cellulose column chromn-atography, QAE-Seph%adex Ediethyl-(2-hydroxypropyl)aminoethyl-Sephadex] column chromatography1 Sephade...

Human hexosaminidase A was covalently bound to soluble poly(N-vinylpyrrolidone), and the effect of this binding on the enzyme inactivation by various procedures was investigated. Whereas the polymer-bound hexosaminidase underwent inactivation to the same extent as the free enzyme, when exposed to heat or acidic pH, the conjugation to polymer appeared to protect the enzyme towards proteolysis. T...