ライフサイエンスコーパス: alpha-galactosidase

1 e expression of aga, the structural gene for alpha-galactosidase.

2 ently hydrolyzed to galactose and glucose by alpha-galactosidase.

3 activity-based probes that target retaining alpha-galactosidases.

4 lage specimens were treated with recombinant alpha-galactosidase (100 U/ml), and the absence of alpha

5 lticenter phase 3 trial of recombinant human alpha -galactosidase A (rh-alpha GalA) replacement in pa

6 urrently, no method is available to identify alpha-galactosidase A (agalA) mutations determining clin

7 sive disorder in which affected persons lack alpha-galactosidase A (alpha -GalA), which leads to exce

8 Fabry disease results from deficient alpha-galactosidase A (alpha-Gal A) activity and the pat

9 -linked lysosomal storage disorder caused by alpha-galactosidase A (alpha-GAL A) deficiency.

10 could lower globotriaosylceramide levels in alpha-galactosidase A (alpha-gal A) knockout mice, a mod

11 used by a deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-gal A).

12 used by a deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-gal A).

13 used by a deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-gal A).

14 metabolic disorder caused by a deficiency of alpha-galactosidase A (alpha-Gal A).

15 c disorder that is caused by a deficiency of alpha-galactosidase A (alpha-Gal A).

16 ith Fabry disease, an X-linked deficiency of alpha-galactosidase A (alpha-Gal A).

17 bry disease is an X-linked inherited loss of alpha-galactosidase A (alpha-Gal A).

18 used by a deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-gal A).

19 sulting from deficient activity of lysosomal alpha-galactosidase A (alpha-Gal A).

20 A deficiency in the lysosomal hydrolase alpha-galactosidase A (alpha-gal A; EC ) leads to impair

21 om mutations in the gene encoding the enzyme alpha-galactosidase A (alpha-gal A; EC ).

22 ed metabolic disorder due to a deficiency of alpha-galactosidase A (alpha-gal A; EC 3.2.1.22).

23 Plasma alpha-galactosidase A (alpha-Gal) was measured in 79 men

24 antigen only accumulates upon inhibition of alpha-galactosidase A (alpha-Gal-A) in lysosomes.

25 Clinical assessments, alpha-galactosidase A (alpha-GalA) activities, glycosphi

26 hat encodes the lysosomal exoglycohydrolase, alpha-galactosidase A (alpha-GalA), cause Fabry disease,

27 em storage disorder due to the deficiency of alpha-galactosidase A (GALA).

28 osomal storage disorder due to deficiency of alpha-galactosidase A (GLA) activity that results in the

29 Alpha-galactosidase A (Gla) deficiency leads to widespre

30 tations in sarcomere protein, PRKAG2, LAMP2, alpha-galactosidase A (GLA), and several mitochondrial g

31 n label extension study of recombinant human alpha-galactosidase A (r-halphaGalA), administered i.v.

32 acement therapy for Fabry disease (deficient alpha-galactosidase A [alpha-Gal A] activity) were perfo

33 sidase A gene knockout (Gla KO) mice have no alpha-galactosidase A activity and progressively accumul

34 , a genetic disorder caused by deficiency of alpha-galactosidase A activity, is associated with CV dy

35 phenotype observed in patients with residual alpha-galactosidase A activity.

36 ation in the skin due to deficient lysosomal alpha-galactosidase A activity.

37 y disease is caused by deficient activity of alpha-galactosidase A and subsequent accumulation of gly

38 rage disease caused by deficient activity of alpha-galactosidase A and the resultant systemic accumul

39 ormal individuals and patients and mice with alpha-galactosidase A deficiency (human Fabry disease).

40 Fabry disease (alpha-galactosidase A deficiency) is a rare, X-linked ly

41 Fabry disease (alpha-galactosidase A deficiency) is an X-linked recessi

42 Fabry's disease, lysosomal alpha-galactosidase A deficiency, results from the progr

43 In 5- to 6-month-old mice with alpha-galactosidase A deficiency, the average GL-3 conce

44 lation in the heart resulting from lysosomal alpha-galactosidase A deficiency.

45 unity, Darmoise et al. report that lysosomal alpha-galactosidase A destroys self-antigens recognized

46 omal storage disorder caused by a deficit in alpha-galactosidase A enzyme activity leading to glycosp

47 Leukocyte alpha-galactosidase A enzyme activity was mildly reduced

48 sorder caused by the absence or reduction of alpha-galactosidase A enzyme activity.

49 increase in the activity of the therapeutic alpha-galactosidase A enzyme after intramuscular adminis

50 alpha-Galactosidase A gene knockout (Gla KO) mice have n

51 istics of the N215S (c.644A>G [p.Asn215Ser]) alpha-galactosidase A gene variant.

52 We studied whether cardio-specific alpha-galactosidase A gene variants are misinterpreted a

53 th X-linked Fabry disease (FD) caused by GLA(alpha-galactosidase A gene) mutations encoding p.D322E (

54 alpha-Galactosidase A genotype N215S does not lead to th

55 dy, 20 of the 29 patients in the recombinant alpha-galactosidase A group (69 percent) had no microvas

56 Patients in the recombinant alpha-galactosidase A group also had decreased microvasc

57 ercent of patients in the former recombinant alpha-galactosidase A group who had biopsies had clearan

58 ), which were more common in the recombinant alpha-galactosidase A group.

59 the safety and effectiveness of recombinant alpha-galactosidase A in a multicenter, randomized, plac

60 hereafter, all patients received recombinant alpha-galactosidase A in an open-label extension study.

61 controlled trial of intravenous infusions of alpha-galactosidase A in patients with Fabry disease dem

62 he finding of a marked decreased activity of alpha-galactosidase A in white blood cells or cultured s

63 t long-term treatment with recombinant human alpha-galactosidase A may halt the progression of vascul

64 Specific alpha-galactosidase A mutations were identified in 17 pa

65 Recombinant alpha-galactosidase A replacement therapy cleared microv

66 cal trials have shown that recombinant human alpha-galactosidase A replacement therapy--the only dise

67 sease is an X-linked lysosomal deficiency of alpha-galactosidase A that results in cellular accumulat

68 a double-blind, placebo-controlled trial of alpha-galactosidase A therapy, the resting regional cere

69 Five months of recombinant human alpha-galactosidase A treatment in the phase 3 trial res

70 Extracellular alpha-galactosidase A was purified from the culture filt

71 zymes cathepsin L, cathepsin A, cathepsin D, alpha-galactosidase A, arylsulfatase A, and alpha-iduron

72 mal storage disorder caused by deficiency of alpha-galactosidase A, resulting in glycosphingolipid ac

73 ted on a recombinant lysosomal enzyme, human alpha-galactosidase A, that contains mannose 6-phosphate

74 A lysosomal enzyme, alpha-galactosidase A, was responsible for the processin

75 isorder caused by a deficiency of the enzyme alpha-galactosidase A, which results in the progressive

76 verstimulation and deletion of iNKT cells in alpha-galactosidase A-deficient (alphaGalA(-/-)) mice (h

77 , globoside, the disialoganglioside, GD3, or alpha-galactosidase A-digested fraction 3 had no effect.

78 to below starting levels, consistent with an alpha-galactosidase A-independent salvage pathway for gl

79 ide (Gb3Cer) in the kidneys of wild-type and alpha-galactosidase A-knockout (Fabry) mice was possible

80 lity and glycolipid accumulation, we studied alpha-galactosidase A-knockout mice or primary cultures

81 Recent studies in the alpha-galactosidase A-knockout mouse suggested that a de

82 ity, associated with eNOS uncoupling, in the alpha-galactosidase A-knockout mouse.

83 percent of patients who received recombinant alpha-galactosidase A.

84 l storage disorder caused by a deficiency of alpha-galactosidase A.

85 abolism resulting from deficient activity of alpha-galactosidase A.

86 ed by deficient activity of lysosomal enzyme alpha-galactosidase A.

87 disease, which is caused by a deficiency in alpha-galactosidase A.

88 vascular tissue secondary to a deficiency in alpha-galactosidase A.

89 n-label extension study of recombinant human alpha-galactosidase A.

90 the GLA gene, which leads to a deficiency in alpha-galactosidase A.

91 sed CGT, GalT2, and GalT6 but did not change alpha-galactosidase activities or mRNA levels.

92 T had greater Gb3 synthase (GalT6) and lower alpha-galactosidase activities than HBEC, whereas lactos

93 The bacteria had an 85% reduction in alpha-galactosidase activity and showed virtually no tra

94 from P(A) results in a 500-fold increase in alpha-galactosidase activity in the cell.

95 The lower HPT alpha-galactosidase activity was associated with reduced

96 The alpha-galactosidase AgaA from the thermophilic microorga

97 zymes acid-beta-glucosidase (GCase) and acid-alpha-galactosidase (alpha-Gal A) hydrolyze the sphingol

98 ne noroviruses (genogroup III) interact with alpha-galactosidase (alpha-Gal) carbohydrates, and murin

99 Deficiency in the lysosomal enzyme alpha-galactosidase (alpha-GAL) causes an accumulation o

100 The enzyme alpha-galactosidase (alpha-GAL, also known as alpha-GAL

101 The human lysosomal enzymes alpha-galactosidase (alpha-GAL, EC 3.2.1.22) and alpha-N

102 Alpha-galactosidase (alpha-Gal; EC 3.2.1.22) is involved

103 lar activities of the lysosomal glycosidases alpha-galactosidase, alpha-mannosidase and neuraminidase

104 obes 3 and 4 inhibit the two human retaining alpha-galactosidases alphaGal A and alphaGal B covalentl

105 has been shown to restore activity of mutant alpha-galactosidase and is currently in clinical trial f

106 n in expression of the raf operon genes aga (alpha-galactosidase) and rafEFG (raffinose substrate bin

107 ase-2, hexosaminidase, galactosylceramidase, alpha-galactosidase, and beta-galactosidase) mediating g

108 ch other by results for raffinose, rhamnose, alpha-galactosidase, and beta-galactosidase: positive, n

109 cible alpha-glucosidase, raffinose-inducible alpha-galactosidase, and cellobiose-inducible beta-gluco

110 ective hydrolysis of RFOs increase the Cicer alpha-galactosidase application in food processing indus

111 2 (mannitol negative and beta galactosidase, alpha galactosidase, beta glucuronidase, and trehalose p

112 annanases, BoMan26A and BoMan26B, and a GH36 alpha-galactosidase, BoGal36A.

113 alpha-Galactosidase caused loss of both L1 structures an

114 tly identified a novel prokaryotic family of alpha-galactosidases (CAZy GH110) with highly restricted

115 s disease, an X-linked disorder of lysosomal alpha-galactosidase deficiency, leads to substrate accum

116 s a rare, sex-linked disorder resulting from alpha-galactosidase deficiency.

117 ial transport of a model therapeutic enzyme (alpha-galactosidase, deficient in lysosomal Fabry diseas

118 d lectins, following treatment of cells with alpha-galactosidase, demonstrate that differentiated cel

119 btained by hydrolyzing native guar gum using alpha-galactosidase enzyme.

120 The subcellular distribution of recombinant alpha-galactosidase expressed from different vectors was

121 ed that 50 of the 67 participants had mutant alpha-galactosidase forms suitable for targeting by miga

122 The initial assay of mutant alpha-galactosidase forms that we used to categorize 67

123 all randomly assigned patients (with mutant alpha-galactosidase forms that were suitable or not suit

124 int analysis, involving patients with mutant alpha-galactosidase forms that were suitable or not suit

125 nd GH36 through biochemical analysis of GH36 alpha-galactosidase from Thermotoga maritima (TmGalA).

126 lysosomal storage disorder caused by loss of alpha-galactosidase function.

127 nhanced GalT6 gene transcription and reduced alpha-galactosidase gene transcription and occur despite

128 ivery of the human coagulation factor IX and alpha-galactosidase genes into endogenous genomic loci w

129 osome-associated membrane protein 2 (LAMP2), alpha-galactosidase (GLA), and acid alpha-1,4-glucosidas

130 saposin (Psap), Niemann Pick type C2 (Npc2), alpha-galactosidase (Gla), are up-regulated in early adi

131 alpha-galactosidase has been shown to reduce gas product

132 tophan residue at position 16 of coffee bean alpha-galactosidase has previously been shown to be esse

133 nnose 6-phosphate measured and the amount of alpha-galactosidase hydrolyzed.

134 To evaluate the efficacy and tolerability of alpha-galactosidase in the treatment of gas-related symp

135 alization of the endogenous activity of both alpha-galactosidases in cell extracts, thereby providing

136 aperone, stabilizes specific mutant forms of alpha-galactosidase, increasing enzyme trafficking to ly

137 Furthermore, a recombinant alpha-galactosidase molecule with a His6 tag at its C-te

138 RNA steady-state levels but no difference in alpha-galactosidase mRNA half-life.

139 osidase activity was associated with reduced alpha-galactosidase mRNA steady-state levels but no diff

140 oss of function observed for the more severe alpha-galactosidase mutants could be enhanced by combini

141 egatively affected by protein aggregation of alpha-galactosidase mutants, revealing a qualitative dif

142 re randomized to receive placebo (n = 25) or alpha-galactosidase (n = 27).

143 Incubation with alpha-galactosidase resulted in complete removal of alph

144 er trials are needed to confirm this result, alpha-galactosidase seems to be a safe, well tolerated a

145 alpha-galactosidase significantly reduced global distres

146 Treatment of cartilage xenografts with alpha-galactosidase successfully removes alpha-gal epito

147 h is in striking contrast to all other known alpha-galactosidases that use a retaining mechanism.

148 Porcine PTs were treated with recombinant alpha-galactosidase to eliminate alpha-gal epitopes and

149 The inflammatory response within the alpha-galactosidase-treated xenografts was much lower th

150 In addition, recombinant alpha-galactosidase was compared to the native enzyme wi

151 Cicer alpha-galactosidase was immobilized onto functionalized

152 A cDNA encoding coffee bean alpha-galactosidase was subcloned into baculovirus expre

153 The expressed protein (recombinant alpha-galactosidase) was immunologically reactive with a

154 synthase (GalT2), Gb3 synthase (GalT6), and alpha-galactosidase were studied in HBECs exposed to TNF

155 s also sensitive for as little as 2.5 microg alpha-galactosidase, which contains 117 pmol mannose 6-p

156 Among patients with suitable mutant alpha-galactosidase who received migalastat for up to 24

157 In search of alpha-galactosidases with improved kinetic properties fo