ライフサイエンスコーパス: beta-hexosaminidase

1 sulfate, but it is a preferred substrate for beta-hexosaminidase.

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

3 oinositide (PI) hydrolysis, and secretion of beta-hexosaminidase.

4 signal transduction defined by exocytosis of beta-hexosaminidase.

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

6 ocyclitols are potent inhibitors of N-acetyl-beta-hexosaminidases.

7 unit for the heterodimeric lysosomal enzyme, beta-hexosaminidase A (alpha beta), as well as for the h

8 beta-Hexosaminidase A (alphabeta) is a heterodimer, wher

9 2 in the brain, is caused by a deficiency of beta-hexosaminidase A (Hex A) or GM2 activator.

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

11 Loss of function of the enzyme beta-hexosaminidase A (HexA) causes the lysosomal storag

12 o participate in the formation of functional beta-hexosaminidase A activity as indicated by activator

13 enerative disorder caused by a deficiency of beta-hexosaminidase A activity.

14 lbeta1-->4Glcbet a1-1'Cer) are refractory to beta-hexosaminidase A and sialidase, respectively, we ha

15 e Neu5Ac of 6'GM2 were readily hydrolyzed by beta-hexosaminidase A and sialidase, respectively, witho

16 opes showed significant co-localization with beta-hexosaminidase A and the azurophilic marker MPO in

17 e is responsible for the metabolic bypass of beta-hexosaminidase A deficiency.

18 expressed human azurophilic granule-resident beta-hexosaminidase A displayed the capacity to generate

19 mbranes and presents them in soluble form to beta-hexosaminidase A for cleavage of N-acetyl-d-galacto

20 activity comparable with that of recombinant beta-hexosaminidase A formed by the co-expression of the

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

22 iency prevents the formation of a functional beta-hexosaminidase A heterodimer resulting in the sever

23 secreted high levels of biologically active beta-hexosaminidase A in vitro and cross-corrected the m

24 Among human isozymes, only beta-hexosaminidase A together with the GM2 activator pr

25 he substrate (GM2) for the defective enzyme (beta-hexosaminidase A) prevents GSL accumulation and the

26 rotein to stimulate the hydrolysis of GM2 by beta-hexosaminidase A, GM2 activator was found to bind a

27 ge diseases that are caused by deficiency of beta-hexosaminidase A, which comprises an alphabeta hete

28 function in the GM2-hydrolyzing activity of beta-hexosaminidase A.

29 but not the hydrolysis of GalNAc from GM2 by beta-hexosaminidase A.

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

31 he oligosaccharide from GM2 was resistant to beta-hexosaminidase A.

32 nes (HEXA and HEXB) encoding the subunits of beta-hexosaminidase A.

33 Accumulation of beta-hexosaminidases A and B substrates is presumed to c

34 cells, where it reduced heparan sulfate and beta-hexosaminidase accumulation to control levels.

35 form is indicative of the action of a novel beta-hexosaminidase activity and suggests a modification

36 ge disorders characterized by the absence of beta-hexosaminidase activity and the accumulation of GM2

37 r glycolipid storage and increased levels of beta-hexosaminidase activity in visceral organs.

38 onal populations, histochemical staining for beta-hexosaminidase activity, a lysosomal enzyme involve

39 nstrated that it cosedimented with lysosomal beta-hexosaminidase activity.

40 ed ecotropic retroviruses encoding the human beta-hexosaminidase alpha-subunit cDNA and transduced mu

41 In humans, beta-hexosaminidase alpha-subunit deficiency prevents th

42 plant, produced substantial amounts of human beta-hexosaminidase alpha-subunit transcript and protein

43 oles were exocytic and mediated secretion of beta-hexosaminidase and cytokines accompanied by Munc13-

44 tent inhibitor than luteolin or cromolyn for beta-hexosaminidase and histamine secretion from LAD2 ce

45 age disorder characterized by the absence of beta-hexosaminidase and storage of G(M2) ganglioside and

46 yt VII inhibit Ca(2+)-triggered secretion of beta-hexosaminidase and surface translocation of Lgp120,

47 lycosaminoglycans are crucial substrates for beta-hexosaminidase and that their lack of storage in Ta

48 MMCs) from SLP76(-/-) mice failed to release beta-hexosaminidase and to secrete IL-6 after FcepsilonR

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

50 hydrolases, e.g., of beta-galactosidase and beta-hexosaminidases, and of GM2-activator protein, caus

51 of these enzymes, suggesting that HYAL1 and beta-hexosaminidase are functionally redundant in HA and

52 The lysosomal beta-hexosaminidases are dimers composed of alpha and be

53 release of the secretory granule constituent beta-hexosaminidase, as well as the generation of the me

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

55 generation of lysosomal iGb3 in mice lacking beta-hexosaminidase b results in severe NKT cell deficie

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

57 t for the intimate involvement of Glu-355 in beta-hexosaminidase B-mediated catalysis.

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

59 extracellular appearance of cathepsin B and beta-hexosaminidase but not cathepsins D or L.

60 a/alpha)(8) barrel topology similar to other beta-hexosaminidases but significant differences exist i

61 We have shown that expression of beta-hexosaminidase by intracranial delivery of recombin

62 ium levels in monocytes induced secretion of beta-hexosaminidase, cathepsins, and myeloperoxidase in

63 rovides in vivo evidence that both HYAL1 and beta-hexosaminidase cleave chondroitin sulfate, but it i

64 is an autosomal recessive disorder caused by beta-hexosaminidase deficiency in which the ganglioside

65 ice deficient in both enzymes, as well as in beta-hexosaminidase-deficient mice, indicating that both

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

67 The importance of beta-hexosaminidase for restricting mycobacterial growth

68 naphylactic release of renin, histamine, and beta-hexosaminidase from mast cells was confirmed in the

69 1 microM when evaluated against the N-acetyl-beta-hexosaminidase from Streptomyces plicatus.

70 d was prevented by rAAV-mediated transfer of beta-hexosaminidase gene function at considerable distan

71 o injected HDAd encoding the beta subunit of beta-hexosaminidase (Hexb) into Hexb-deficient mice, a m

72 y tmIgE has been confirmed by the release of beta-hexosaminidase in a cell-to-cell contact assay usin

73 normalization of brain glycosaminoglycan and beta-hexosaminidase in MPS I mice 5 mo after moderate ye

74 Neonatal restitution of beta-hexosaminidase in mutant mice by gene therapy succe

75 a total deficiency of all forms of lysosomal beta-hexosaminidase including the small amount of beta-h

76 pharmacologic agents inhibited exocytosis of beta-hexosaminidase induced by SCF or cross-linked IgE.

77 cellular staining pattern and the release of beta-hexosaminidase into the cytosol, apoE4-transfected

78 GM2 gangliosidosis caused by a deficiency in beta-hexosaminidase, is characterized by progressive neu

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

80 infections was confirmed in macrophages from beta-hexosaminidase knockout mice.

81 umber of secondary accumulations in neurons [beta-hexosaminidase, LAMP1(lysosome-associated membrane

82 NSCs also increased brain beta-hexosaminidase levels, reduced ganglioside storage

83 EB activation also rescues the activity of a beta-hexosaminidase mutant associated with the developme

84 ellular chitinase, a specific chitoporin, or beta-hexosaminidases, nor did they exhibit chemotaxis, t

85 elop new potent inhibitors of human N-acetyl-beta-hexosaminidases, particularly when combined with th

86 g and flow cytometry; function by release of beta-hexosaminidase, PGD(2), leukotriene C(4) (LTC(4)),

87 Histological analysis revealed beta-hexosaminidase-positive cells in the central nervou

88 the generated libraries with human N-acetyl-beta-hexosaminidases produced only moderate inhibitory a

89 Release of beta-hexosaminidase, prostaglandin D2, and GM-CSF and ch

90 ead, a transient attenuation of IgE-mediated beta-hexosaminidase release and cytokine production was

91 ionomycin-induced degranulation, as shown by beta-hexosaminidase release assays.

92 The mutant proteins induced less beta-hexosaminidase release from mast cells than the wil

93 AM induced histamine or beta-hexosaminidase release from rat and human MCs throu

94 fic IgE antibodies was assessed by measuring beta-hexosaminidase release from rat basophilic leukaemi

95 hibitor LY294002, reduced agonist-stimulated beta-hexosaminidase release in a dose-dependent manner.

96 y human IgE and antigens, as demonstrated by beta-hexosaminidase release in vitro and passive cutaneo

97 by the requirement of the FYB SH3 domain in beta-hexosaminidase release, but not adhesion, and the u

98 ly, that irradiation did not directly induce beta-hexosaminidase release.

99 d by Fc gammaRII/III was not associated with beta-hexosaminidase release.

100 heritable deficiency of a lysosomal enzyme, beta-hexosaminidase, results in the storage of the enzym

101 hexosaminidase including the small amount of beta-hexosaminidase S present in the Sandhoff disease mo

102 histamine inhibited carbachol (CCh)-induced beta-hexosaminidase secretion and prevented the formatio

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

104 t as illustrated by its ability to stimulate beta-hexosaminidase secretion from primary rabbit lacrim

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

106 and dose-dependent reductions of CCh-induced beta-hexosaminidase secretion.

107 l glycosphingolipid biosynthesis inhibitors (beta-hexosaminidase substrate inhibitors) were combined

108 nt adeno-associated viral vectors expressing beta-hexosaminidase subunits (rAAV2/1-Hex).

109 To identify the domains of the human beta-hexosaminidase subunits that determine substrate sp

110 We confirm that Lyn(-/-) BMMCs release more beta-hexosaminidase than wild-type BMMCs following Fceps

111 easuring the release of the lysosomal enzyme beta-hexosaminidase, the appearance on the plasma membra

112 endoglycosidase HYAL1 and the exoglycosidase beta-hexosaminidase to the lysosomal degradation of HA.

113 Ala)), were unable to target cathepsin D and beta-hexosaminidase to the lysosome.

114 Beta-hexosaminidase was characterized as a peptidoglycan

115 ined by RNAi depletion, the lysosomal enzyme beta-hexosaminidase was identified as an important facto

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

117 tered, widespread and abundant expression of beta-hexosaminidase with consequent clearance of glycoco