ライフサイエンスコーパス: acid beta-glucosidase

1 ts specific and multivalent interaction with acid beta-glucosidase.

2 correlated with their activation effects on acid beta-glucosidase.

3 ficient activity of the lysosomal hydrolase, acid beta-glucosidase.

4 the cell from adverse hydrolytic activity of acid beta-glucosidase.

5 ough the salvage pathway involving, in part, acid beta-glucosidase 1 (GBA1), which cleaves glucosylce

6 In the present study, we examined whether acid beta-glucosidase 1 (GBA1), which hydrolyzes glucosy

7 isease resulting from a defect in the enzyme acid beta-glucosidase 1.

8 posin B and C localized the neurotrophic and acid beta-glucosidase activation properties to the carbo

9 CD-/- mice resulted in cellular decreases of acid beta-glucosidase activity and protein.

10 rase at dose of 5 U/kg bodyweight normalised acid beta-glucosidase activity of splenic Gaucher's cell

11 rapid neurodegenerative course had two novel acid beta-glucosidase alleles: a complex, maternally der

12 Conduritol B epoxide is an inhibitor of acid beta-glucosidase, and lowers glucosylceramide degra

13 The x-ray structure of N370S mutant acid beta-glucosidase at acidic and neutral pH values in

14 saposin C binding and further enhancement of acid beta-glucosidase catalytic activity.

15 The finding of neutralizing antibodies to acid beta-glucosidase during enzyme therapy for Gaucher

16 A loss in lysosomal acid-beta-glucosidase enzyme (GCase) activity due to bia

17 rder, results from the deficient activity of acid beta-glucosidase (GBA).

18 The lysosomal acid beta-glucosidase GBA1 and the non-lysosomal beta-gl

19 Isofagomine (IFG) is an acid beta-glucosidase (GCase) active site inhibitor that

20 (LSD), is caused by insufficient activity of acid beta-glucosidase (GCase) and the resultant glucosyl

21 Inherited deficiency of acid beta-glucosidase (GCase) due to biallelic mutations

22 Gaucher disease is caused by defective acid beta-glucosidase (GCase) function.

23 Defective lysosomal acid beta-glucosidase (GCase) in Gaucher disease causes

24 ll model (CBE-N2a) was created by inhibiting acid beta-glucosidase (GCase) in N2a cells with condurit

25 Acid beta-glucosidase (GCase) is a 497-amino acid, membr

26 inherited disease caused by mutations at the acid beta-glucosidase (GCase) locus (GBA).

27 lysosomal sphingolipid degradation pathway, acid beta-glucosidase (GCase) requires saposin C for opt

28 disease is caused by mutations in the enzyme acid beta-glucosidase (GCase), the most common of which

29 GBA1 gene that encodes the lysosomal enzyme acid beta-glucosidase (GCase).

30 d glucosylceramide (GC) cleavage activity by acid beta-glucosidase (GCase).

31 Human lysosomal enzymes acid-beta-glucosidase (GCase) and acid-alpha-galactosida

32 ptomatic patients with Gaucher disease (GD) (acid beta-glucosidase [Gcase] deficiency) are treated wi

33 ubtypes, all resulting from mutations in the acid beta-glucosidase gene.

34 rrelations and functional domains within the acid beta-glucosidase gene.

35 e disorder caused by deficiency in lysosomal acid beta-glucosidase (GlcCerase), the enzyme responsibl

36 The acid beta-glucosidase (glucocerbrosidase (GCase)) bindin

37 or for the hydrolysis of glucosylceramide by acid beta-glucosidase in mammals.

38 nactivated the wild-type, but not the N370S, acid beta-glucosidase in vitro.

39 l B epoxide, a potent inhibitor of lysosomal acid beta-glucosidase, inhibited pyridoxine-beta-D-gluco

40 CD spectral changes indicated saposin C and acid beta-glucosidase interaction only in the presence o

41 Acid beta-glucosidase is a lysosomal membrane protein th

42 A conformational change of acid beta-glucosidase is shown to accompany activity rec

43 orage disease, is caused by mutations at the acid beta-glucosidase locus.

44 ally distinct subtypes result from different acid beta-glucosidase mutations encoding enzymes with ab

45 Inhibition of acid beta-glucosidase promoted faster axonal elongation

46 These studies show that acid beta-glucosidase requires interfaces composed of NC

47 Periodic infusions of macrophage-targeted acid beta-glucosidase reverse hepatosplenomegaly, hemato

48 therapy developed neutralizing antibodies to acid beta-glucosidase that were associated with a lack o

49 , carrying mutation(s) in GBA, which encodes acid beta-glucosidase, were recruited at the SZMC Gauche

50 d minor allergic reactions and antibodies to acid beta-glucosidase within the first 6 months of treat