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mast cell granule beta-hexosaminidase (显示 HEXA 蛋白) is crucial for defense against bacterial invasion, but is not involved in the allergic response. the bactericidal mechanism of beta-hexosaminidase (显示 HEXA 蛋白) 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 (显示 HEXA 蛋白) activity.
Data suggest that pathogenesis of Sandhoff disease (heritable beta-hexosaminidase (显示 HEXA 蛋白) deficiency) involves an increase in macrophage-inflammatory protein 1alpha (显示 CCL3 蛋白) that induces monocytes to infiltrate the CNS and trigger neuronal apoptosis.
The bicistronic beta-hexosaminidase (显示 HEXA 蛋白) 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 (显示 HEXA 蛋白) 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 (显示 CYB5D2 蛋白) 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 (显示 HHEX 蛋白) and its isoenzymes HEX A and HEX (显示 HHEX 蛋白) 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 (显示 POLG 蛋白) that confer GM2 (显示 CYB5D2 蛋白) 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 (显示 CD4 蛋白)(+)T cells because knock-down of HEXA (显示 HEXA 蛋白) or HEXB gene causes a loss of function of i-DCs.
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 (显示 CYB5D2 蛋白) gangliosidosis is caused by the gene mutation. (review)
A highly significant correlation of HEX (显示 HHEX 蛋白)-7 and %CDT has been found. Because of exclusion of the P isoform, HEX (显示 HHEX 蛋白)-7 could be a useful supplementary marker for detecting chronic alcohol abuse.
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