戻る
「早戻しボタン」を押すと検索画面に戻ります。 [閉じる]

コーパス検索結果 (１語後でソート)

通し番号をクリックするとPubMedの該当ページを表示します
1 age disorder caused by deficiency of alpha-l-iduronidase.
2 r characterized by the deficiency of alpha-L-iduronidase.
3 tic deficiencies in IDUA, coding for alpha-l-iduronidase.
4 ene-corrected with virally delivered alpha-L-iduronidase.
5 usions of low-dose recombinant human alpha-l-iduronidase.
6 caused by a deficiency of the enzyme alpha-L-iduronidase.
7 -galactosidase A, arylsulfatase A, and alpha-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 t also had a transient increase in leukocyte iduronidase activity to the lower normal range.
15                Twenty weeks after treatment, iduronidase activity was increased in visceral organs of
16 02X showed a significant increase in alpha-L-iduronidase activity when cultured in the presence of ge
17 ct approached mid-normal levels of leukocyte iduronidase activity with no evidence of genome editing.
18 aluations, measurements of leukocyte alpha-L-iduronidase activity, and urinary glycosaminoglycan excr
19 in the restoration of 2.8% of normal alpha-L-iduronidase activity.
20 n = 24) heterozygous for the mutated alpha-L-iduronidase allele (carriers unaffected by the storage d
21 ate receptor-mediated uptake because alpha-l-iduronidase and alpha-glucosidase induced tolerance with
22 isorder resulting from deficiency of alpha-L-iduronidase and lysosomal accumulation of glycosaminogly
23 ed by deficiency of lysosomal enzyme alpha-L-iduronidase, and patients treated with allogeneic HSCT a
24                            Dephospho-alpha-L-iduronidase-aptamer conjugate was taken up in saturable
25 a-galactosidase, beta-glucuronidase, alpha-L-iduronidase, aryl sulfatase, and galactose-6-sulfate sul
26  to 22 years) with recombinant human alpha-L-iduronidase at a dose of 125,000 U per kilogram of body
27  the oligosaccharides of recombinant alpha-L-iduronidase at each of its six N-glycosylation sites.
28        All canines received i.v. recombinant iduronidase at the FDA-approved human dose or a higher d
29 rug failed to change baseline plasma alpha-L-iduronidase but did increase leukocyte alpha-L-iduronida
30 nducing antigen-specific immune tolerance to iduronidase could improve the effectiveness of recombina
31 olysaccharidosis IIIB, MPS IIIB) and alpha-l-iduronidase deficiency (MPS I) are heritable lysosomal s
32  was taken up in saturable manner by alpha-L-iduronidase-deficient mouse fibroblasts, with half-maxim
33 lysosomal storage in canines and humans with iduronidase-deficient MPS I, but therapy usually also in
34 ereas ovalbumin and dephosphorylated alpha-l-iduronidase did not.
35 rong antibody response to the enzyme alpha-l-iduronidase during enzyme replacement therapy of a canin
36 disease caused by loss of the enzyme alpha-L-iduronidase (encoded by the IDUA gene), which participat
37 four patients deficient in leukocyte alpha-L-iduronidase enzyme activity (median age, 1.8 years; rang
38 MDI at follow-up study and leukocyte alpha-L-iduronidase enzyme activity (P = .02).
39 nor with homozygous normal leukocyte alpha-L-iduronidase enzyme activity.
40                             A normal alpha-l-iduronidase enzyme level obtained post-HCT was another h
41 as conjugated to a lysosomal enzyme, alpha-l-iduronidase, from which mannose 6-phosphate had been rem
42  values, including rs6856425 tagging alpha-l-iduronidase (IDUA) (P = 2.1 x 10(-5), after Bonferroni c
43 zyme that incorporates peptides from alpha-l-iduronidase (IDUA) and apolipoprotein E II (APO) to enha
44 mal storage disorders resulting from alpha-L-iduronidase (IDUA) deficiency and iduronate-2-sulfatase
45 ate (HS) accumulation resulting from alpha-l-iduronidase (Idua) deficiency.
46  frame-shift insertion affecting the alpha-L-iduronidase (IDUA) gene (c.19_20insCGGCCCCC), a mutation
47 c deficiency of the lysosomal enzyme alpha-l-iduronidase (IDUA), exhibit accumulation of glycosaminog
48 sing the same approach, we expressed alpha-L-iduronidase (IDUA), the defective enzyme in Mucopolysacc
49                  A lysosomal enzyme, alpha-L-iduronidase (IDUA), was used for biological and therapeu
50 ld overexpress the lysosomal enzyme, alpha-l-iduronidase (IDUA), which is deficient in patients with
51 ycans due to a genetic deficiency of alpha-L-iduronidase (IDUA), which results in progressive systemi
52 ed by mutations in the gene encoding alpha-L-iduronidase (IDUA), which ultimately causes toxic buildu
53 cells (HSPCs) transduced ex vivo with an a-L-iduronidase (IDUA)-encoding lentiviral vector after myel
54 or production of a lysosomal enzyme, alpha-L-iduronidase (IDUA).
55 avoid these sugars, the human enzyme alpha-L-iduronidase (IDUA, EC 3.2.1.76), with a C-terminal ER-re
56 thesis of substrates for the enzymes alpha-l-iduronidase, iduronate-2-sulfatase, and N-acetylgalactos
57               Therefore, immune tolerance to iduronidase improved the efficacy of enzyme replacement
58 iation of ERT with recombinant human alpha-L-iduronidase--improved enzyme uptake in organs.
59  failed to change baseline leukocyte alpha-L-iduronidase in a phase 1/2 trial.
60  enzyme replacement therapy with recombinant iduronidase in canine MPS I and could potentially improv
61 ement therapy with recombinant human alpha-L-iduronidase in patients with this disorder.
62                                      alpha-L-Iduronidase is a lysosomal hydrolase that is deficient i
63                    Recombinant human alpha-L-iduronidase, isolated from secretions of an overexpressi
64 drome) is a congenital deficiency of alpha-L-iduronidase, leading to lysosomal storage of glycosamino
65 sions of the human lysosomal enzymes alpha-l-iduronidase or acid alpha-glucosidase with the receptor-
66  canines with MPS I were either tolerized to iduronidase or left nontolerant.
67 tment produced a similar increase in alpha-L-iduronidase protein in Hurler cells.
68                     Determination of alpha-L-iduronidase protein levels by an immunoquantification as
69  I, treatment with recombinant human alpha-L-iduronidase reduces lysosomal storage in the liver and a
70               Nontolerized canines developed iduronidase-specific antibodies that proportionally redu
71 o understand the potential impact of alpha-L-iduronidase-specific antibodies, we studied whether indu
72 ement therapy with recombinant human alpha-L-iduronidase successfully reduces lysosomal storage in ca
73  proteins comprised of RTB and human alpha-L-iduronidase, the corrective enzyme for Mucopolysaccharid
74 RISPR-Cas9 that targets the lysosomal enzyme iduronidase to the CCR5 safe harbor locus in human CD34+
75 uronidase but did increase leukocyte alpha-L-iduronidase to within the normal range.
76 uld improve the effectiveness of recombinant iduronidase treatment in canines.
77 ibodies that proportionally reduced in vitro iduronidase uptake.
78                        We found that alpha-L-iduronidase was not required for stem cell renewal, and
79                  Serum antibodies to alpha-L-iduronidase were detected in four patients.
80 I (MPS I) involves i.v. injection of alpha-l-iduronidase, which can be taken up by cells throughout t