ライフサイエンスコーパス: iduronidase

1 tic deficiencies in IDUA, coding for alpha-l-iduronidase.

2 ene-corrected with virally delivered alpha-L-iduronidase.

3 usions of low-dose recombinant human alpha-l-iduronidase.

4 age disorder caused by deficiency of alpha-l-iduronidase.

5 caused by a deficiency of the enzyme alpha-L-iduronidase.

6 -galactosidase A, arylsulfatase A, and alpha-iduronidase.

7 r characterized by the deficiency of alpha-L-iduronidase.

8 by mutations in idua, which encodes alpha-L-iduronidase, a gene addition strategy to prevent, and po

9 dies, leukocyte 4 methylumbelliferyl-alpha-L-iduronidase activities in this kindred were as follows:

10 himerism and normal peripheral-blood alpha-L-iduronidase activity (event-free survival rate, 85 perce

11 Cord-blood donors had normal alpha-L-iduronidase activity (mean number of cells, 10.53x10(7)

12 natal BMT was effective at restoring alpha-l-iduronidase activity and clearing elevated glycosaminogl

13 Both the alpha-L-iduronidase activity and protein level resulting from th

14 Twenty weeks after treatment, iduronidase activity was increased in visceral organs of

15 02X showed a significant increase in alpha-L-iduronidase activity when cultured in the presence of ge

16 aluations, measurements of leukocyte alpha-L-iduronidase activity, and urinary glycosaminoglycan excr

17 in the restoration of 2.8% of normal alpha-L-iduronidase activity.

18 n = 24) heterozygous for the mutated alpha-L-iduronidase allele (carriers unaffected by the storage d

19 ate receptor-mediated uptake because alpha-l-iduronidase and alpha-glucosidase induced tolerance with

20 isorder resulting from deficiency of alpha-L-iduronidase and lysosomal accumulation of glycosaminogly

21 ed by deficiency of lysosomal enzyme alpha-L-iduronidase, and patients treated with allogeneic HSCT a

22 Dephospho-alpha-L-iduronidase-aptamer conjugate was taken up in saturable

23 a-galactosidase, beta-glucuronidase, alpha-L-iduronidase, aryl sulfatase, and galactose-6-sulfate sul

24 to 22 years) with recombinant human alpha-L-iduronidase at a dose of 125,000 U per kilogram of body

25 the oligosaccharides of recombinant alpha-L-iduronidase at each of its six N-glycosylation sites.

26 All canines received i.v. recombinant iduronidase at the FDA-approved human dose or a higher d

27 nducing antigen-specific immune tolerance to iduronidase could improve the effectiveness of recombina

28 olysaccharidosis IIIB, MPS IIIB) and alpha-l-iduronidase deficiency (MPS I) are heritable lysosomal s

29 was taken up in saturable manner by alpha-L-iduronidase-deficient mouse fibroblasts, with half-maxim

30 lysosomal storage in canines and humans with iduronidase-deficient MPS I, but therapy usually also in

31 ereas ovalbumin and dephosphorylated alpha-l-iduronidase did not.

32 rong antibody response to the enzyme alpha-l-iduronidase during enzyme replacement therapy of a canin

33 disease caused by loss of the enzyme alpha-L-iduronidase (encoded by the IDUA gene), which participat

34 four patients deficient in leukocyte alpha-L-iduronidase enzyme activity (median age, 1.8 years; rang

35 MDI at follow-up study and leukocyte alpha-L-iduronidase enzyme activity (P = .02).

36 nor with homozygous normal leukocyte alpha-L-iduronidase enzyme activity.

37 A normal alpha-l-iduronidase enzyme level obtained post-HCT was another h

38 as conjugated to a lysosomal enzyme, alpha-l-iduronidase, from which mannose 6-phosphate had been rem

39 values, including rs6856425 tagging alpha-l-iduronidase (IDUA) (P = 2.1 x 10(-5), after Bonferroni c

40 mal storage disorders resulting from alpha-L-iduronidase (IDUA) deficiency and iduronate-2-sulfatase

41 ate (HS) accumulation resulting from alpha-l-iduronidase (Idua) deficiency.

42 c deficiency of the lysosomal enzyme alpha-l-iduronidase (IDUA), exhibit accumulation of glycosaminog

43 A lysosomal enzyme, alpha-L-iduronidase (IDUA), was used for biological and therapeu

44 ld overexpress the lysosomal enzyme, alpha-l-iduronidase (IDUA), which is deficient in patients with

45 or production of a lysosomal enzyme, alpha-L-iduronidase (IDUA).

46 avoid these sugars, the human enzyme alpha-L-iduronidase (IDUA, EC 3.2.1.76), with a C-terminal ER-re

47 thesis of substrates for the enzymes alpha-l-iduronidase, iduronate-2-sulfatase, and N-acetylgalactos

48 Therefore, immune tolerance to iduronidase improved the efficacy of enzyme replacement

49 iation of ERT with recombinant human alpha-L-iduronidase--improved enzyme uptake in organs.

50 enzyme replacement therapy with recombinant iduronidase in canine MPS I and could potentially improv

51 ement therapy with recombinant human alpha-L-iduronidase in patients with this disorder.

52 alpha-L-Iduronidase is a lysosomal hydrolase that is deficient i

53 Recombinant human alpha-L-iduronidase, isolated from secretions of an overexpressi

54 drome) is a congenital deficiency of alpha-L-iduronidase, leading to lysosomal storage of glycosamino

55 sions of the human lysosomal enzymes alpha-l-iduronidase or acid alpha-glucosidase with the receptor-

56 canines with MPS I were either tolerized to iduronidase or left nontolerant.

57 tment produced a similar increase in alpha-L-iduronidase protein in Hurler cells.

58 Determination of alpha-L-iduronidase protein levels by an immunoquantification as

59 I, treatment with recombinant human alpha-L-iduronidase reduces lysosomal storage in the liver and a

60 Nontolerized canines developed iduronidase-specific antibodies that proportionally redu

61 o understand the potential impact of alpha-L-iduronidase-specific antibodies, we studied whether indu

62 ement therapy with recombinant human alpha-L-iduronidase successfully reduces lysosomal storage in ca

63 proteins comprised of RTB and human alpha-L-iduronidase, the corrective enzyme for Mucopolysaccharid

64 uld improve the effectiveness of recombinant iduronidase treatment in canines.

65 ibodies that proportionally reduced in vitro iduronidase uptake.

66 We found that alpha-L-iduronidase was not required for stem cell renewal, and

67 Serum antibodies to alpha-L-iduronidase were detected in four patients.

68 I (MPS I) involves i.v. injection of alpha-l-iduronidase, which can be taken up by cells throughout t