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mast cell granule beta-hexosaminidase is crucial for defense against bacterial invasion, but is not involved in the allergic response. the bactericidal mechanism of beta-hexosaminidase involves degradation of bacterial cell wall peptidoglycan.
transgenic inducible strains of Sandhoff disease mice provide a dynamic platform with which to explore the pathophysiological sequelae immediately after loss of neuronal lysosomal beta-hexosaminidase activity.
Thymic involution and corticosterone level in Sandhoff disease model mice.
These data suggest that the restricted ketogenic diet and N-butyldeoxynojirimycin may be a potential combinatorial therapy for Sandhoff disease.
Data suggest that pathogenesis of Sandhoff disease (heritable beta-hexosaminidase deficiency) involves an increase in macrophage-inflammatory protein 1alpha that induces monocytes to infiltrate the CNS and trigger neuronal apoptosis.
The bicistronic beta-hexosaminidase vector can reverse the biochemical defects and down-stream consequences in Sandhoff neurons, reinforcing its potential for Sandhoff disease in vivo gene therapy.
Beta-hexosaminidase is a peptidoglycan hydrolase that surprisingly exerts its mycobactericidal effect at the macrophage plasma membrane during mycobacteria-induced secretion of lysosomes
There was no change in the level of GM2 storage and pro-apoptotic activity or astrocyte activation in HexB-/- knockout mice
Mannose receptor (-/-) liver sinusoidal endothelial cells had markedly and significantly reduced enzyme activities for four out of five lysosomal enzymes tested, i.e., cathepsin-D, alpha-mannosidase, beta-hexosaminidase and arylsulfatase.
Reported data present, for the first time, reference values for urinary activities of HEX and its isoenzymes HEX A and HEX B in children and adolescent.
a modified human hexosaminidase subunit beta (HexB), which we have termed mod2B, composed of homodimeric beta subunits that contain amino acid sequences from the alpha subunit that confer GM2 ganglioside-degrading activity and protease resistance.
Mutations of the HEXB gene is associated with maple syrup urine disease or Sandhoff disease.
report on the heterogeneity of the mutational spectrum of the HEXB gene in Indian patients with Sandhoff disease
The absence of beta-N-acetyl-hexosaminidase activity does not alter the differentiation of i-DCs from HSCs, but it is critical for the activation of CD4(+)T cells because knock-down of HEXA or HEXB gene causes a loss of function of i-DCs.
Concentration and specific activity of N-acetyl-B-hexosaminidase in palatine tonsils in patients with tonsillar hypertrophy and chronic tonsillitis both in childhood and adulthood significantly increase in comparison to healthy individuals.
A total of 19 HEXB variants were found in the 1092 genomes of which 5 are suspected of having a deleterious effect on hexosaminidase activity.
DNA from Iranian Tay-Sachs patients reveals a novel mutation in HEXB predicting a termination codon or nonsense mutation.
A patient with Sandhoff disease also is found to have a compound macro-deletion in HEXB.
GM2 gangliosidosis is caused by the gene mutation. (review)
A highly significant correlation of HEX-7 and %CDT has been found. Because of exclusion of the P isoform, HEX-7 could be a useful supplementary marker for detecting chronic alcohol abuse.
Expression of beta-hexosaminidase in the neurons of Sandhoff disease patients rescues transgenic mice from neurodegeneration.
minigene studies revealed the presence of a novel alternative spliced HEXB mRNA variant also present in normal cells
Characterization of seven novel mutations on the HEXB gene in French Sandhoff patients.
identified 27 different mutations, 14 of which were novel, in the HEXA gene and 14 different mutations, 8 of which unreported until now, in the HEXB gene, and attempted to correlate these mutations with the clinical presentation of the patients
We describe a novel HEXB mutation that is shared among 4 patients with Sandhoff disease.
The non-random distribution of plasma membrane-associated beta-hexosaminidase and beta-galactosidase and their localization within lipid microdomains, suggest a role of these enzymes in the local reorganization of glycosphingolipid-based signalling units.
Plasma beta-hexosaminidase and beta-galactosidase) levels are higher in patients with Alzheimer's disease-type 2 diabetes mellitus (T2DM) compared to those with T2DM alone.
Down-regulation of beta-N-acetyl-D-glucosaminidase increases Akt1 activity in thyroid anaplastic cancer cells
Urinary NAG (ninefold), NGAL (1.5-fold), and H-FABP (3.5-fold) were significantly elevated in normoalbuminuric diabetic patients compared with nondiabetic control subjects.
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 hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II).
N-acetyl-beta-glucosaminidase subunit beta
, beta-N-acetylhexosaminidase subunit beta
, beta-hexosaminidase subunit beta
, hexosaminidase subunit B
, cervical cancer proto-oncogene 7 protein
, beta-N-acetylhexosaminidase beta subunit
, beta-hexosaminidase beta subunit
, 65 kDa epididymal boar protein