Detalhe da pesquisa
1.
Duvoglustat HCl Increases Systemic and Tissue Exposure of Active Acid α-Glucosidase in Pompe Patients Co-administered with Alglucosidase α.
Mol Ther
; 25(5): 1199-1208, 2017 05 03.
Artigo
em Inglês
| MEDLINE | ID: mdl-28341561
2.
Coformulation of a Novel Human α-Galactosidase A With the Pharmacological Chaperone AT1001 Leads to Improved Substrate Reduction in Fabry Mice.
Mol Ther
; 23(7): 1169-1181, 2015 Jul.
Artigo
em Inglês
| MEDLINE | ID: mdl-25915924
3.
Co-administration with the pharmacological chaperone AT1001 increases recombinant human α-galactosidase A tissue uptake and improves substrate reduction in Fabry mice.
Mol Ther
; 20(4): 717-26, 2012 Apr.
Artigo
em Inglês
| MEDLINE | ID: mdl-22215019
4.
Smooth muscle cell alpha2delta-1 subunits are essential for vasoregulation by CaV1.2 channels.
Circ Res
; 105(10): 948-55, 2009 Nov 06.
Artigo
em Inglês
| MEDLINE | ID: mdl-19797702
5.
The pharmacological chaperone 1-deoxygalactonojirimycin reduces tissue globotriaosylceramide levels in a mouse model of Fabry disease.
Mol Ther
; 18(1): 23-33, 2010 Jan.
Artigo
em Inglês
| MEDLINE | ID: mdl-19773742
6.
Improved efficacy of a next-generation ERT in murine Pompe disease.
JCI Insight
; 4(5)2019 03 07.
Artigo
em Inglês
| MEDLINE | ID: mdl-30843882
7.
Partial restoration of the keratocyte phenotype to bovine keratocytes made fibroblastic by serum.
Invest Ophthalmol Vis Sci
; 43(11): 3416-21, 2002 Nov.
Artigo
em Inglês
| MEDLINE | ID: mdl-12407151
8.
The pharmacological chaperone AT2220 increases the specific activity and lysosomal delivery of mutant acid alpha-glucosidase, and promotes glycogen reduction in a transgenic mouse model of Pompe disease.
PLoS One
; 9(7): e102092, 2014.
Artigo
em Inglês
| MEDLINE | ID: mdl-25036864
9.
The pharmacological chaperone AT2220 increases recombinant human acid α-glucosidase uptake and glycogen reduction in a mouse model of Pompe disease.
PLoS One
; 7(7): e40776, 2012.
Artigo
em Inglês
| MEDLINE | ID: mdl-22815812
10.
The pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of beta-glucosidase.
FEBS J
; 277(7): 1618-38, 2010 Apr.
Artigo
em Inglês
| MEDLINE | ID: mdl-20148966