miglustat has been researched along with Acid Alpha-Glucosidase Deficiency in 9 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 1 (11.11) | 29.6817 |
| 2010's | 5 (55.56) | 24.3611 |
| 2020's | 3 (33.33) | 2.80 |
| Authors | Studies |
|---|---|
| Cobucci-Ponzano, B; D'Alonzo, D; De Fenza, M; Guaragna, A; Huebecker, M; Iacono, R; Moracci, M; Palumbo, G; Parenti, G; Platt, F; Porto, C; Priestman, DA | 1 |
| Fleet, GWJ; Jia, YM; Kanekiyo, U; Kato, A; Kishida, M; Li, YX; Lu, TT; Nakagome, I; Nash, RJ; Shinzawa, K; Tanaka, N; Yoshida, T; Yoshimura, K; Yu, CY | 1 |
| Bratkovic, D; Byrne, BJ; Castelli, J; Díaz-Manera, J; Goldman, M; Jiang, H; Kishnani, PS; Kuchipudi, S; Laforêt, P; Mozaffar, T; Roberts, M; Schoser, B; Sitaraman, S; Toscano, A; van der Ploeg, AT | 1 |
| Blair, HA | 1 |
| Do, HV; Feng, J; Frascella, M; Garcia, A; Gotschall, R; Khanna, R; Lun, Y; Martina, JA; Nair, A; Ponery, AS; Puertollano, R; Raben, N; Ralston, E; Schilling, A; Soska, R; Tuske, S; Valenzano, KJ; Valle, MCD; Xu, S | 1 |
| Agovino, T; Andria, G; Ascione, S; Danesino, C; De Filippi, P; Della Casa, R; Donati, MA; Fecarotta, S; la Marca, G; Mollica, C; Morandi, LO; Nusco, E; Ombrone, D; Parenti, G; Pasanisi, MB; Pichiecchio, A; Porto, C; Ravaglia, S; Romano, A; Rosa, M; Rossi, B; Sacchini, M | 1 |
| Andreotti, G; Cubellis, MV; Monticelli, M | 1 |
| Andria, G; Barone, MV; Cardone, M; Fontana, F; Parenti, G; Porto, C; Rossi, B; Tarallo, A; Tuzzi, MR | 1 |
| Aoki, K; Eto, Y; Ida, H; Kaneshiro, E; Kawagoe, S; Kobayashi, H; Ohashi, T; Shimada, Y; Shimizu, H | 1 |
1 review(s) available for miglustat and Acid Alpha-Glucosidase Deficiency
| Article | Year |
|---|---|
Cipaglucosidase Alfa: First Approval.
Topics: 1-Deoxynojirimycin; Adult; Enzyme Replacement Therapy; Glycogen; Glycogen Storage Disease Type II; Humans | 2023 |
1 trial(s) available for miglustat and Acid Alpha-Glucosidase Deficiency
| Article | Year |
|---|---|
Safety and efficacy of cipaglucosidase alfa plus miglustat versus alglucosidase alfa plus placebo in late-onset Pompe disease (PROPEL): an international, randomised, double-blind, parallel-group, phase 3 trial.
Topics: 1-Deoxynojirimycin; Adolescent; alpha-Glucosidases; Double-Blind Method; Glycogen Storage Disease Type II; Humans; Treatment Outcome | 2021 |
7 other study(ies) available for miglustat and Acid Alpha-Glucosidase Deficiency
| Article | Year |
|---|---|
N-Butyl-l-deoxynojirimycin (l-NBDNJ): Synthesis of an Allosteric Enhancer of α-Glucosidase Activity for the Treatment of Pompe Disease.
Topics: 1-Deoxynojirimycin; Allosteric Regulation; alpha-Glucosidases; Cell Line; Enzyme Activation; Enzyme Inhibitors; Fibroblasts; Glycogen Storage Disease Type II; Humans; Lysosomes; Models, Molecular; Stereoisomerism | 2017 |
5-
Topics: 1-Deoxynojirimycin; Alkylation; alpha-Glucosidases; Enzyme Inhibitors; Fibroblasts; Glycogen Storage Disease Type II; Humans; Molecular Dynamics Simulation; Molecular Structure; Mutation; Protein Conformation; Protein Stability; Recombinant Proteins | 2022 |
Improved efficacy of a next-generation ERT in murine Pompe disease.
Topics: 1-Deoxynojirimycin; alpha-Glucosidases; Animals; Disease Models, Animal; Enzyme Replacement Therapy; Female; Glycogen; Glycogen Storage Disease Type II; Humans; Lysosomes; Male; Mannosephosphates; Mice; Mice, Knockout; Muscle, Skeletal; Rats; Rats, Sprague-Dawley | 2019 |
A chaperone enhances blood α-glucosidase activity in Pompe disease patients treated with enzyme replacement therapy.
Topics: 1-Deoxynojirimycin; Adolescent; Adult; alpha-Glucosidases; Animals; Child; Child, Preschool; Disease Models, Animal; Dried Blood Spot Testing; Drug Synergism; Enzyme Replacement Therapy; Enzyme Stability; Female; Glycogen Storage Disease Type II; Glycoside Hydrolase Inhibitors; Humans; Male; Mice; Middle Aged; Young Adult | 2014 |
Looking for protein stabilizing drugs with thermal shift assay.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Drug Evaluation, Preclinical; Enzyme Stability; Fabry Disease; Glycogen Storage Disease Type II; High-Throughput Screening Assays; Humans; Temperature | 2015 |
The pharmacological chaperone N-butyldeoxynojirimycin enhances enzyme replacement therapy in Pompe disease fibroblasts.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Animals; Biological Transport; Blotting, Western; Cell Line; Drug Stability; Enzyme Inhibitors; Fibroblasts; Glycogen Storage Disease Type II; Humans; Lysosomes; Mice; Microscopy, Confocal | 2009 |
Endoplasmic reticulum stress induces autophagy through activation of p38 MAPK in fibroblasts from Pompe disease patients carrying c.546G>T mutation.
Topics: 1-Deoxynojirimycin; alpha-Glucosidases; Autophagy; Cells, Cultured; DNA Mutational Analysis; Endoplasmic Reticulum Stress; Enzyme Activation; Enzyme Activators; Fibroblasts; Glycogen Storage Disease Type II; MAP Kinase Signaling System; p38 Mitogen-Activated Protein Kinases; Phosphoproteins; Phosphorylation; Point Mutation; Protein Folding; Protein Isoforms; Unfolded Protein Response | 2011 |