ライフサイエンスコーパス: hexosaminidase

1 olysis by exoglycosidases (galactosidase and hexosaminidase).

2 l transduction defined by exocytosis of beta-hexosaminidase.

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

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

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

6 ed with release of the soluble mediator 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 f O-GlcNAcase and is distinct from lysosomal hexosaminidases.

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

13 invertebrates due to the action of specific hexosaminidases.

14 of selectivity versus long OGA and lysosomal hexosaminidases.

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

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

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

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

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

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

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

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

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

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

25 1-->4Glcbet a1-1'Cer) are refractory to beta-hexosaminidase A and sialidase, respectively, we have re

26 5Ac of 6'GM2 were readily hydrolyzed by beta-hexosaminidase A and sialidase, respectively, without GM

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

55 racellular glycosidase activities, including hexosaminidase activity.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

74 Using hexosaminidase assay, we determined that EF24 inhibits p

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

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

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

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

79 .5% of these activities were associated with hexosaminidase B and the intermediate isozyme fractions.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

106 Given hexosaminidases' important roles in osteoarthritis, we d

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

166 Beta-hexosaminidase was characterized as a peptidoglycan hydr

167 Hexosaminidase was found to be the dominant enzyme relea

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

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

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

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