ライフサイエンスコーパス: hexosaminidase

1 olysis by exoglycosidases (galactosidase and hexosaminidase).

2 ed with release of the soluble mediator beta-hexosaminidase.

3 l transduction defined by exocytosis of beta-hexosaminidase.

4 se, and minimally sensitive to beta-N-acetyl hexosaminidase.

5 endosomal/lysosomal markers LAMP-1 and beta-hexosaminidase.

6 te, but it is a preferred substrate for beta-hexosaminidase.

7 eads to MPR missorting and hypersecretion of hexosaminidase.

8 asing activity of the Fused lobes processing hexosaminidase.

9 hosphate receptors and enhanced secretion of hexosaminidase.

10 itide (PI) hydrolysis, and secretion of beta-hexosaminidase.

11 (-1)) that are similar to those of lysosomal hexosaminidases.

12 f O-GlcNAcase and is distinct from lysosomal hexosaminidases.

13 ed Glu-355, a highly conserved residue among hexosaminidases.

14 ve belonged to the large GH20 family of beta-hexosaminidases.

15 invertebrates due to the action of specific hexosaminidases.

16 of selectivity versus long OGA and lysosomal hexosaminidases.

17 itols are potent inhibitors of N-acetyl-beta-hexosaminidases.

18 eta-domain is related to bacterial and human hexosaminidases.

19 for the heterodimeric lysosomal enzyme, beta-hexosaminidase A (alpha beta), as well as for the homodi

20 beta-Hexosaminidase A (alphabeta) is a heterodimer, whereas h

21 the brain, is caused by a deficiency of beta-hexosaminidase A (Hex A) or GM2 activator.

22 atabolism of GM2 to GM3 in man requires beta-hexosaminidase A (HexA) and a protein cofactor, the GM2

23 We identify hexosaminidase A (HEXA) as an NAFLD-induced hepatokine i

24 Loss of function of the enzyme beta-hexosaminidase A (HexA) causes the lysosomal storage dis

25 eurological disorder caused by deficiency of hexosaminidase A (HexA).

26 ticipate in the formation of functional beta-hexosaminidase A activity as indicated by activator-depe

27 tive disorder caused by a deficiency of beta-hexosaminidase A activity.

28 1-->4Glcbet a1-1'Cer) are refractory to beta-hexosaminidase A and sialidase, respectively, we have re

29 5Ac of 6'GM2 were readily hydrolyzed by beta-hexosaminidase A and sialidase, respectively, without GM

30 showed significant co-localization with beta-hexosaminidase A and the azurophilic marker MPO in human

31 The ganglioside GM2 is processed by beta-hexosaminidase A and when non-functional GM2 accumulates

32 responsible for the metabolic bypass of beta-hexosaminidase A deficiency.

33 ssed human azurophilic granule-resident beta-hexosaminidase A displayed the capacity to generate pauc

34 es and presents them in soluble form to beta-hexosaminidase A for cleavage of N-acetyl-d-galactosamin

35 ity comparable with that of recombinant beta-hexosaminidase A formed by the co-expression of the alph

36 , various strategies aimed at restoring beta-hexosaminidase A have been explored.

37 prevents the formation of a functional beta-hexosaminidase A heterodimer resulting in the severe neu

38 eted high levels of biologically active beta-hexosaminidase A in vitro and cross-corrected the metabo

39 Among human isozymes, only beta-hexosaminidase A together with the GM2 activator protein

40 The % hexosaminidase A values as derived from the ratio betwee

41 bstrate (GM2) for the defective enzyme (beta-hexosaminidase A) prevents GSL accumulation and the neur

42 sulfated substrates were highly specific for hexosaminidase A, and in fractionated serum, cells, and

43 n to stimulate the hydrolysis of GM2 by beta-hexosaminidase A, GM2 activator was found to bind avidly

44 complex, which interacts with the hydrolase Hexosaminidase A, the enzyme that cleaves the terminal s

45 seases that are caused by deficiency of beta-hexosaminidase A, which comprises an alphabeta heterodim

46 IRMAb-enzyme fusion protein is preserved for hexosaminidase A, which is mutated in Tay Sachs disease,

47 conversion of ganglioside GM2 to GM3 by beta-hexosaminidase A.

48 igosaccharide from GM2 was resistant to beta-hexosaminidase A.

49 HEXA and HEXB) encoding the subunits of beta-hexosaminidase A.

50 tion in the GM2-hydrolyzing activity of beta-hexosaminidase A.

51 ot the hydrolysis of GalNAc from GM2 by beta-hexosaminidase A.

52 Accumulation of beta-hexosaminidases A and B substrates is presumed to cause

53 We identified beta-hexosaminidase, a conserved enzyme across commensals of

54 6 significantly enriched pathways, including hexosaminidase-A deficiency, and multiple porphyric diso

55 s, where it reduced heparan sulfate and beta-hexosaminidase accumulation to control levels.

56 is indicative of the action of a novel beta-hexosaminidase activity and suggests a modification in t

57 sorders characterized by the absence of beta-hexosaminidase activity and the accumulation of GM2 gang

58 in a selective increase of the extracellular hexosaminidase activity and, to a lesser degree, of the

59 colipid storage and increased levels of beta-hexosaminidase activity in visceral organs.

60 Hexosaminidase activity throughout the brain and spinal

61 creased ceramide, glucosylceramide, GM3, and hexosaminidase activity were also found in SOD1(G93A) mi

62 populations, histochemical staining for beta-hexosaminidase activity, a lysosomal enzyme involved in

63 e fluid, reduced eosinophil peroxidase and B-hexosaminidase activity, increased airway width, improve

64 racellular glycosidase activities, including hexosaminidase activity.

65 ted that it cosedimented with lysosomal beta-hexosaminidase activity.

66 otropic retroviruses encoding the human beta-hexosaminidase alpha-subunit cDNA and transduced multipo

67 In humans, beta-hexosaminidase alpha-subunit deficiency prevents the for

68 , produced substantial amounts of human beta-hexosaminidase alpha-subunit transcript and protein, whi

69 Cell proliferation was assessed by hexosaminidase and 3-(4,5-dimethylthiazol 2-yl)-2,5-diph

70 were exocytic and mediated secretion of beta-hexosaminidase and cytokines accompanied by Munc13-4 dif

71 inhibitor than luteolin or cromolyn for beta-hexosaminidase and histamine secretion from LAD2 cells s

72 The following enzymes were analyzed: hexosaminidase and its isoenzyme A, N-acetyl-alpha-D-glu

73 isorder characterized by the absence of beta-hexosaminidase and storage of G(M2) ganglioside and rela

74 I inhibit Ca(2+)-triggered secretion of beta-hexosaminidase and surface translocation of Lgp120, wher

75 aminoglycans are crucial substrates for beta-hexosaminidase and that their lack of storage in Tay-Sac

76 Based upon their relative resistance to both hexosaminidase and to in vitro galactosylation, O-GlcNAc

77 from SLP76(-/-) mice failed to release beta-hexosaminidase and to secrete IL-6 after FcepsilonRI cro

78 derived membranes (FBM) was measured by beta-hexosaminidase and tryptase release.

79 olases, e.g., of beta-galactosidase and beta-hexosaminidases, and of GM2-activator protein, cause inf

80 hese enzymes, suggesting that HYAL1 and beta-hexosaminidase are functionally redundant in HA and chon

81 The lysosomal beta-hexosaminidases are dimers composed of alpha and beta su

82 NamZ-like peptidoglycan hexosaminidases are mainly present in the phylum Bactero

83 se of the secretory granule constituent beta-hexosaminidase, as well as the generation of the membran

84 Using hexosaminidase assay, we determined that EF24 inhibits p

85 ha beta), as well as for the homodimers beta-hexosaminidase B (beta beta) and S (alpha alpha).

86 dase A (alphabeta) is a heterodimer, whereas hexosaminidase B (betabeta) and S (alphaalpha) are homod

87 e mutations in the MutL homolog 1 (MLH1) and hexosaminidase B (HEXB) genes, respectively.

88 e 3 (Neu3), beta-galactosidase 1 (Glb1), and hexosaminidase B (HexB), possess hydrolytic activities t

89 .5% of these activities were associated with hexosaminidase B and the intermediate isozyme fractions.

90 sease, a lysosomal storage disease caused by hexosaminidase B deficiency.

91 ation of lysosomal iGb3 in mice lacking beta-hexosaminidase b results in severe NKT cell deficiency,

92 lsulfatase (As-1), thrombin receptor (Cf2r), hexosaminidase b(Hexb), 3-hydroxy-3-methylglutaryl coenz

93 ethod (except for subjects with thermolabile hexosaminidase B) and may be helpful in genotype determi

94 was used to identify the active site of beta-hexosaminidase B, a beta-subunit dimer.

95 sease, including a patient with thermolabile hexosaminidase B, had less than 2% of noncarrier activit

96 the intimate involvement of Glu-355 in beta-hexosaminidase B-mediated catalysis.

97 We hypothesized that increasing beta-hexosaminidase (beta-hex) activity would lead to a reduc

98 GCF myeloperoxidase (MPO), beta-N-acetyl-hexosaminidase (beta-NAH), and beta-glucuronidase (beta-

99 ysis of myeloperoxidase (MPO), beta-N-acetyl-hexosaminidase (beta-NAH), cathepsin D (CD), and elastas

100 acellular appearance of cathepsin B and beta-hexosaminidase but not cathepsins D or L.

101 ha)(8) barrel topology similar to other beta-hexosaminidases but significant differences exist in the

102 We have shown that expression of beta-hexosaminidase by intracranial delivery of recombinant a

103 in vitro by using functional bioassays (beta-hexosaminidase, calcium flux, and chemokine synthesis) a

104 evels in monocytes induced secretion of beta-hexosaminidase, cathepsins, and myeloperoxidase in the e

105 es in vivo evidence that both HYAL1 and beta-hexosaminidase cleave chondroitin sulfate, but it is a p

106 autosomal recessive disorder caused by beta-hexosaminidase deficiency in which the ganglioside GM2 a

107 eficient in both enzymes, as well as in beta-hexosaminidase-deficient mice, indicating that both enzy

108 ogies to the alpha- and beta-chains of human hexosaminidase despite their marked differences in subst

109 signatures, improves behavior, restores beta-hexosaminidase enzymatic activity and Hexb expression, p

110 a deficiency in the beta subunit of the beta-hexosaminidase enzyme (Hexb).

111 is due to functional redundancy in the beta-hexosaminidase enzyme system.

112 activities revealed that even at neutral pH, hexosaminidase expressed a measurable activity, much hig

113 The importance of beta-hexosaminidase for restricting mycobacterial growth duri

114 lactic release of renin, histamine, and beta-hexosaminidase from mast cells was confirmed in the effl

115 using a thioglycoligase derived from a GH20 hexosaminidase from Streptomyces plicatus in which the c

116 roM when evaluated against the N-acetyl-beta-hexosaminidase from Streptomyces plicatus.

117 (glucocerebrosidase-1, glucocerebrosidase-2, hexosaminidase, galactosylceramidase, alpha-galactosidas

118 prevented by rAAV-mediated transfer of beta-hexosaminidase gene function at considerable distances f

119 XB vector can restore central nervous system hexosaminidase (Hex) enzyme activity, decrease GM2 level

120 Previously, we have identified two hexosaminidases (HEX-2 and HEX-3) in C. elegans, which p

121 ected HDAd encoding the beta subunit of beta-hexosaminidase (Hexb) into Hexb-deficient mice, a model

122 and time-regulated process that blocks beta-hexosaminidase, impacting membrane and cytoskeletal move

123 Given hexosaminidases' important roles in osteoarthritis, we d

124 gE has been confirmed by the release of beta-hexosaminidase in a cell-to-cell contact assay using hum

125 arrow mast cells blocked the release of beta-hexosaminidase in an Ag-specific fashion and prevented a

126 ng the critical role of myeloid-derived beta-hexosaminidase in maintaining neuronal health and establ

127 lization of brain glycosaminoglycan and beta-hexosaminidase in MPS I mice 5 mo after moderate yet sus

128 Neonatal restitution of beta-hexosaminidase in mutant mice by gene therapy successful

129 ited release of the secretory granule marker hexosaminidase in response to carbachol but not to antig

130 e of Hexb, encoding the beta subunit of beta-hexosaminidase, in both mice and patients with neurodege

131 al deficiency of all forms of lysosomal beta-hexosaminidase including the small amount of beta-hexosa

132 potent inhibitors of long OGA and lysosomal hexosaminidases, including PUGNAc and NAG-thiazoline, su

133 acologic agents inhibited exocytosis of beta-hexosaminidase induced by SCF or cross-linked IgE.

134 lar staining pattern and the release of beta-hexosaminidase into the cytosol, apoE4-transfected cells

135 neuronal homeostasis, here we show that beta-hexosaminidase is secreted by microglia and integrated i

136 angliosidosis caused by a deficiency in beta-hexosaminidase, is characterized by progressive neurodeg

137 -like immunoreactivity in the brains of beta-hexosaminidase knock-out (HEXB KO) mice.

138 tions was confirmed in macrophages from beta-hexosaminidase knockout mice.

139 of secondary accumulations in neurons [beta-hexosaminidase, LAMP1(lysosome-associated membrane prote

140 sensitized mice, resulted in increased beta-hexosaminidase levels in serum and BAL compared to sham-

141 NSCs also increased brain beta-hexosaminidase levels, reduced ganglioside storage and d

142 sidic N-glycans generated by the activity of hexosaminidases located in the apoplast/plasma membrane.

143 es), platelet factor IV (alpha granules), or hexosaminidase (lysosomes) between null and wild-type pl

144 imal velocity of 15 pmol of tyrosine/unit of hexosaminidase/min.

145 tivation also rescues the activity of a beta-hexosaminidase mutant associated with the development of

146 ns, the sensitivity of the C. elegans double hexosaminidase mutant was assessed.

147 ar chitinase, a specific chitoporin, or beta-hexosaminidases, nor did they exhibit chemotaxis, transp

148 new potent inhibitors of human N-acetyl-beta-hexosaminidases, particularly when combined with the str

149 flow cytometry; function by release of beta-hexosaminidase, PGD(2), leukotriene C(4) (LTC(4)), IL-5,

150 Histological analysis revealed beta-hexosaminidase-positive cells in the central nervous sys

151 generated libraries with human N-acetyl-beta-hexosaminidases produced only moderate inhibitory activi

152 Release of beta-hexosaminidase, prostaglandin D2, and GM-CSF and changes

153 Moreover, removal of O-GlcNAc by hexosaminidase reduced AMPK activity.

154 e (PTK) phosphorylation, Ca++ flux, and beta-hexosaminidase release (i.e., degranulation).

155 a transient attenuation of IgE-mediated beta-hexosaminidase release and cytokine production was obser

156 ycin-induced degranulation, as shown by beta-hexosaminidase release assays.

157 The mutant proteins induced less beta-hexosaminidase release from mast cells than the wild-typ

158 AM induced histamine or beta-hexosaminidase release from rat and human MCs through a

159 gE antibodies was assessed by measuring beta-hexosaminidase release from rat basophilic leukaemia cel

160 or LY294002, reduced agonist-stimulated beta-hexosaminidase release in a dose-dependent manner.

161 an IgE and antigens, as demonstrated by beta-hexosaminidase release in vitro and passive cutaneous an

162 ized platelet exocytosis assay, we show that hexosaminidase release is stimulated by either Ca(++) or

163 timuli (Ca(++) or GTP-gamma-S) serotonin and hexosaminidase release requires the same membrane fusion

164 uction) but not degranulation (histamine and hexosaminidase release).

165 he requirement of the FYB SH3 domain in beta-hexosaminidase release, but not adhesion, and the up-reg

166 though nicotine did not significantly affect hexosaminidase release, IgG, or methacholine-induced air

167 ast cells from A3AR(-/-) mice as measured by hexosaminidase release.

168 Fc gammaRII/III was not associated with beta-hexosaminidase release.

169 hat irradiation did not directly induce beta-hexosaminidase release.

170 t that lysosomal glycosidases, in particular hexosaminidase, represent a distinct subset of cartilage

171 esent work addresses the contribution of the hexosaminidase responsible for removing O-GlcNAc (ie, O-

172 table deficiency of a lysosomal enzyme, beta-hexosaminidase, results in the storage of the enzyme's s

173 aminidase including the small amount of beta-hexosaminidase S present in the Sandhoff disease model m

174 off disease [SD]) genes, with subsequent low hexosaminidase(s) activity.

175 phages by 100 nM C3a, (b) inhibition of beta-hexosaminidase secretion (IC(50) 8 nM) from human LAD2 m

176 amine inhibited carbachol (CCh)-induced beta-hexosaminidase secretion and prevented the formation of

177 Histamine and 5-HT acutely stimulated beta-hexosaminidase secretion at lower, but not higher, conce

178 illustrated by its ability to stimulate beta-hexosaminidase secretion from primary rabbit lacrimal gl

179 ural selectivity and potency as observed for hexosaminidase secretion in mast cells.

180 Net and vectorial beta-hexosaminidase secretion, cytosolic Ca(2+) (Ca(i)) eleva

181 ose-dependent reductions of CCh-induced beta-hexosaminidase secretion.

182 In a mouse model of colitis, beta-hexosaminidase-specific lymphocytes protected against in

183 cosphingolipid biosynthesis inhibitors (beta-hexosaminidase substrate inhibitors) were combined with

184 uring homeostasis and disease and identified hexosaminidase subunit beta (Hexb) as a stably expressed

185 Here, we produced a modified human hexosaminidase subunit beta (HexB), which we have termed

186 eno-associated viral vectors expressing beta-hexosaminidase subunits (rAAV2/1-Hex).

187 To identify the domains of the human beta-hexosaminidase subunits that determine substrate specifi

188 onfirm that Lyn(-/-) BMMCs release more beta-hexosaminidase than wild-type BMMCs following FcepsilonR

189 of the N-acetylglucosaminyltransferase or a hexosaminidase that could remove N-acetylglucosamine fro

190 ing the release of the lysosomal enzyme beta-hexosaminidase, the appearance on the plasma membrane of

191 microglia deliver the lysosomal enzyme beta-hexosaminidase to neurons for the degradation of the gan

192 lycosidase HYAL1 and the exoglycosidase beta-hexosaminidase to the lysosomal degradation of HA.

193 , were unable to target cathepsin D and beta-hexosaminidase to the lysosome.

194 Beta-hexosaminidase was characterized as a peptidoglycan hydr

195 Hexosaminidase was found to be the dominant enzyme relea

196 by RNAi depletion, the lysosomal enzyme beta-hexosaminidase was identified as an important factor in

197 ed by IgE cross-linking, the release of beta-hexosaminidase was reduced to about 20% by CE.

198 talytic machinery similar to other family 20 hexosaminidases which cleave beta(1,4)-linked N-acetylgl

199 , widespread and abundant expression of beta-hexosaminidase with consequent clearance of glycoconjuga