Detalhe da pesquisa
1.
Carnitine is a pharmacological allosteric chaperone of the human lysosomal α-glucosidase.
J Enzyme Inhib Med Chem;
36(1): 2068-2079, 2021 Dec.
Artigo
em Inglês
| MEDLINE
| ID: mdl-34565280
2.
A chaperone enhances blood α-glucosidase activity in Pompe disease patients treated with enzyme replacement therapy.
Mol Ther;
22(11): 2004-12, 2014 Nov.
Artigo
em Inglês
| MEDLINE
| ID: mdl-25052852
3.
Pharmacological enhancement of α-glucosidase by the allosteric chaperone N-acetylcysteine.
Mol Ther;
20(12): 2201-11, 2012 Dec.
Artigo
em Inglês
| MEDLINE
| ID: mdl-22990675
4.
Enzyme replacement therapy in patients with Fabry disease: state of the art and review of the literature.
Mol Genet Metab;
107(3): 267-75, 2012 Nov.
Artigo
em Inglês
| MEDLINE
| ID: mdl-22963910
5.
Synergy between the pharmacological chaperone 1-deoxygalactonojirimycin and the human recombinant alpha-galactosidase A in cultured fibroblasts from patients with Fabry disease.
J Inherit Metab Dis;
35(3): 513-20, 2012 May.
Artigo
em Inglês
| MEDLINE
| ID: mdl-22187137
6.
Correction of oxidative stress enhances enzyme replacement therapy in Pompe disease.
EMBO Mol Med;
13(11): e14434, 2021 11 08.
Artigo
em Inglês
| MEDLINE
| ID: mdl-34606154
7.
The pharmacological chaperone N-butyldeoxynojirimycin enhances enzyme replacement therapy in Pompe disease fibroblasts.
Mol Ther;
17(6): 964-71, 2009 Jun.
Artigo
em Inglês
| MEDLINE
| ID: mdl-19293774
8.
The pharmacological chaperone 1-deoxynojirimycin increases the activity and lysosomal trafficking of multiple mutant forms of acid alpha-glucosidase.
Hum Mutat;
30(12): 1683-92, 2009 Dec.
Artigo
em Inglês
| MEDLINE
| ID: mdl-19862843
9.
Synergy between the pharmacological chaperone 1-deoxygalactonojirimycin and agalsidase alpha in cultured fibroblasts from patients with Fabry disease.
J Inherit Metab Dis;
37(1): 145-6, 2014 Jan.
Artigo
em Inglês
| MEDLINE
| ID: mdl-23974650
10.
Pharmacological enhancement of mutated alpha-glucosidase activity in fibroblasts from patients with Pompe disease.
Mol Ther;
15(3): 508-14, 2007 Mar.
Artigo
em Inglês
| MEDLINE
| ID: mdl-17213836
11.
Pharmacological Enhancement of Mutated α-Glucosidase Activity in Fibroblasts from Patients with Pompe Disease.
Mol Ther;
15(3): 508-514, 2007 Mar.
Artigo
em Inglês
| MEDLINE
| ID: mdl-28182897
12.
N-Butyl-l-deoxynojirimycin (l-NBDNJ): Synthesis of an Allosteric Enhancer of α-Glucosidase Activity for the Treatment of Pompe Disease.
J Med Chem;
60(23): 9462-9469, 2017 12 14.
Artigo
em Inglês
| MEDLINE
| ID: mdl-29112434
13.
A novel GLA mutation in a Fabry family with glucose-6-phosphate dehydrogenase deficiency.
J Nephrol;
25(4): 582-5, 2012.
Artigo
em Inglês
| MEDLINE
| ID: mdl-22307442
14.
Abnormal mannose-6-phosphate receptor trafficking impairs recombinant alpha-glucosidase uptake in Pompe disease fibroblasts.
Pathogenetics;
1(1): 6, 2008 Dec 01.
Artigo
em Inglês
| MEDLINE
| ID: mdl-19046416