1-deoxynojirimycin has been researched along with globotriaosylceramide in 21 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 6 (28.57) | 29.6817 |
| 2010's | 14 (66.67) | 24.3611 |
| 2020's | 1 (4.76) | 2.80 |
| Authors | Studies |
|---|---|
| Abe, A; Arend, LJ; Brady, RO; Lee, L; Lingwood, C; Shayman, JA | 1 |
| Abe, A; Brady, RO; Gregory, S; Killen, PD; Kulkarni, A; Lee, L; Shayman, JA | 1 |
| Fan, JQ; Ishii, S; Kulkarni, AB; Mannen, K; Yoshioka, H | 1 |
| Roth, J; Yam, GH; Zuber, C | 1 |
| Falguières, T; Jarvis, RM; Johannes, L; Lord, JM; Platt, FM; Roberts, LM; Sillence, DJ; Smith, DC | 1 |
| Etou, Y | 1 |
| Benjamin, ER; Brignol, N; Desnick, RJ; Feng, J; Frascella, M; Khanna, R; Lockhart, DJ; Lun, Y; Pellegrino, L; Sitaraman, SA; Soska, R; Valenzano, KJ; Young, B | 1 |
| Furukawa, K; Hamanaka, R; Ishii, S; Kulkarni, AB; Kunieda, T; Mannen, K; Matsuda, J; Noguchi, Y; Shiozuka, C; Taguchi, A; Uchio-Yamada, K; Yano, S; Yokoyama, S; Yoshioka, H | 1 |
| Acampora, E; Andria, G; Avolio, V; Gagliardo, C; la Marca, G; Materazzi, S; Parenti, G; Pisani, A; Porto, C; Rosa, M; Tuzzi, MR; Visciano, B | 1 |
| Benjamin, ER; Boudes, P; Brignol, N; Chang, HH; Fuller, M; Germain, DP; Giugliani, R; Hughes, DA; Khanna, R; Lockhart, DJ; Mehta, A; Nicholls, K; Sitaraman, SA; Soska, R; Valenzano, KJ; Young-Gqamana, B | 1 |
| Butters, T; Deepak, S; Hall, CJ; Isenberg, DA; Jury, EC; Magee, AI; McDonald, G; Miguel, L | 1 |
| Drury, JE; García Fernández, JM; Higaki, K; Johnson, JL; Lieberman, RL; Mellet, CO; Mena-Barragán, T; Nakasone, N; Nanba, E; Ninomiya, H; Ohno, K; Sakuraba, H; Suzuki, Y; Tsukimura, T; Yu, Y | 1 |
| Amartino, H; Banikazemi, M; Barlow, C; Barth, J; Benjamin, ER; Bichet, DG; Bratkovic, D; Castelli, J; Charrow, J; Dasouki, M; Ezgu, F; Feldt-Rasmussen, U; Feliciani, C; Finegold, D; Germain, DP; Giraldo, P; Giugliani, R; Goker-Alpan, O; Hughes, DA; Johnson, F; Jovanovic, A; Kirk, J; Lockhart, DJ; Longo, N; Lourenco, CM; Ludington, E; Nedd, K; Nicholls, K; Packman, S; Schiffmann, R; Scott, CR; Shankar, SP; Sharaf El Din, U; Skuban, N; Torra, R; Tuffaha, A; Viereck, C; Waldek, S; Wilcox, WR; Yu, J | 1 |
| Barth, JA; Benjamin, ER; Castelli, JP; Mauer, M; Najafian, B; Sokolovskiy, A; Williams, HN | 1 |
| Ambros, JT; Andrés, A; Bernabéu, AÁ; de Dios García Díaz, J; de Juan-Rivera, J; Del Pino, M; Fernández, E; Fernández, IM; Hernández, D; Luño, J; Paniagua, J; Posada de la Paz, M; Rodríguez-Pérez, JC; Santamaría, R; Torra, R; Torregrosa, JV; Vidau, P | 1 |
| Barisoni, L; Barth, JA; Bichet, DG; Castelli, JP; Colvin, RB; Feldt-Rasmussen, U; Giugliani, R; Holdbrook, F; Hughes, DA; Jennette, JC; Jovanovic, A; Mulberg, A; Nicholls, K; Schiffmann, R; Shankar, SP; Skuban, N | 1 |
| Garzotti, M; Groenen, PMA; Morand, O; Mühlemann, A; Probst, MR; Rickert, V; Üçeyler, N; Welford, RWD | 1 |
| McCafferty, EH; Scott, LJ | 1 |
| Auray-Blais, C; Ballmaier, PJ; Boutin, M; Brand, E; Brand, SM; Lenders, M; Niemietz, C; Schmidt, H; Schmitz, B; Stappers, F; Zibert, A | 1 |
| Mignani, R | 1 |
| Herrmann, K; Jovanovic, A; Vardarli, I; Weidemann, F | 1 |
4 review(s) available for 1-deoxynojirimycin and globotriaosylceramide
| Article | Year |
|---|---|
[Recent therapeutics for Fabry disease].
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Cell- and Tissue-Based Therapy; Fabry Disease; Genetic Therapy; Glucosamine; Humans; Isoenzymes; Mass Screening; Trihexosylceramides | 2009 |
Fabry Nephropathy: An Evidence-Based Narrative Review.
Topics: 1-Deoxynojirimycin; Enzyme Replacement Therapy; Fabry Disease; Female; Galactosidases; Humans; Kidney Diseases; Male; Trihexosylceramides | 2018 |
Migalastat: A Review in Fabry Disease.
Topics: 1-Deoxynojirimycin; Adolescent; Adult; Aged; Aged, 80 and over; Dose-Response Relationship, Drug; Drug Approval; Enzyme Replacement Therapy; Fabry Disease; Female; Glycolipids; Humans; Male; Middle Aged; Mutation; Sphingolipids; Trihexosylceramides | 2019 |
Chaperone Therapy in Fabry Disease.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Fabry Disease; Humans; Male; Mutation; Time-to-Treatment; Trihexosylceramides | 2022 |
2 trial(s) available for 1-deoxynojirimycin and globotriaosylceramide
| Article | Year |
|---|---|
Treatment of Fabry's Disease with the Pharmacologic Chaperone Migalastat.
Topics: 1-Deoxynojirimycin; Adolescent; Adult; Aged; alpha-Galactosidase; Diarrhea; Double-Blind Method; Fabry Disease; Female; Glomerular Filtration Rate; Heart Ventricles; Humans; Hypertrophy, Left Ventricular; Kidney; Male; Middle Aged; Mutation; Trihexosylceramides; Ultrasonography; Young Adult | 2016 |
Migalastat improves diarrhea in patients with Fabry disease: clinical-biomarker correlations from the phase 3 FACETS trial.
Topics: 1-Deoxynojirimycin; Adolescent; Adult; Aged; Biomarkers; Diarrhea; Fabry Disease; Female; Humans; Kidney; Male; Middle Aged; Mutation; Trihexosylceramides; Young Adult | 2018 |
15 other study(ies) available for 1-deoxynojirimycin and globotriaosylceramide
| Article | Year |
|---|---|
Glycosphingolipid depletion in fabry disease lymphoblasts with potent inhibitors of glucosylceramide synthase.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; B-Lymphocytes; Bacterial Toxins; Cell Line, Transformed; Dose-Response Relationship, Drug; Enzyme Inhibitors; Fabry Disease; Fluorescein-5-isothiocyanate; Fluorescent Dyes; Genetic Vectors; Glucosyltransferases; Glycosphingolipids; Herpesvirus 4, Human; Humans; Neutral Glycosphingolipids; Propanolamines; Pyrrolidines; Shiga Toxin 1; Trihexosylceramides | 2000 |
Reduction of globotriaosylceramide in Fabry disease mice by substrate deprivation.
Topics: 1-Deoxynojirimycin; Animals; Dose-Response Relationship, Drug; Fabry Disease; Kidney; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Propanolamines; Pyrrolidines; Trihexosylceramides | 2000 |
Transgenic mouse expressing human mutant alpha-galactosidase A in an endogenous enzyme deficient background: a biochemical animal model for studying active-site specific chaperone therapy for Fabry disease.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Animals; Binding Sites; Cells, Cultured; Disease Models, Animal; Fabry Disease; Fibroblasts; Genetic Therapy; Heart; Humans; Mice; Mice, Knockout; Mice, Transgenic; Molecular Chaperones; Mutation; Myocardium; Protein Folding; RNA, Messenger; Sensitivity and Specificity; Trihexosylceramides | 2004 |
A synthetic chaperone corrects the trafficking defect and disease phenotype in a protein misfolding disorder.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Amino Acid Substitution; Animals; Arginine; Cells, Cultured; Fabry Disease; Fibroblasts; Glutamine; Humans; Lysosomes; Mice; Mice, Transgenic; Molecular Chaperones; Mutation; Phenotype; Protein Binding; Protein Folding; Protein Transport; Trihexosylceramides | 2005 |
The association of Shiga-like toxin with detergent-resistant membranes is modulated by glucosylceramide and is an essential requirement in the endoplasmic reticulum for a cytotoxic effect.
Topics: 1-Deoxynojirimycin; Animals; Cell Death; Cell Line, Tumor; Cell Membrane; Chlorocebus aethiops; Detergents; Endoplasmic Reticulum; Glucosylceramides; Glycosphingolipids; HeLa Cells; Humans; Intracellular Membranes; Proteasome Inhibitors; Protein Transport; Shiga Toxin 1; Trihexosylceramides; Vero Cells | 2006 |
The pharmacological chaperone 1-deoxygalactonojirimycin reduces tissue globotriaosylceramide levels in a mouse model of Fabry disease.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Animals; Blotting, Western; Disease Models, Animal; Fabry Disease; Humans; Immunohistochemistry; Mice; Mice, Inbred C57BL; Mice, Knockout; Mice, Transgenic; Trihexosylceramides | 2010 |
Increased globotriaosylceramide levels in a transgenic mouse expressing human alpha1,4-galactosyltransferase and a mouse model for treating Fabry disease.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Animals; Crosses, Genetic; Disease Models, Animal; Enzyme Activation; Fabry Disease; Female; Galactosyltransferases; Humans; Kidney; Liver; Mice; Mice, Knockout; Mice, Transgenic; Molecular Chaperones; Spleen; Trihexosylceramides; Up-Regulation | 2011 |
Synergy between the pharmacological chaperone 1-deoxygalactonojirimycin and the human recombinant alpha-galactosidase A in cultured fibroblasts from patients with Fabry disease.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Case-Control Studies; Exons; Fabry Disease; Fibroblasts; Genotype; Humans; Lysosomes; Male; Microscopy, Confocal; Microscopy, Fluorescence; Mutation; Recombinant Proteins; Trihexosylceramides | 2012 |
Migalastat HCl reduces globotriaosylsphingosine (lyso-Gb3) in Fabry transgenic mice and in the plasma of Fabry patients.
Topics: 1-Deoxynojirimycin; Administration, Oral; alpha-Galactosidase; Animals; Fabry Disease; Glycolipids; Humans; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Mutation; Reproducibility of Results; Sphingolipids; Sphingosine; Trihexosylceramides | 2013 |
Normalizing glycosphingolipids restores function in CD4+ T cells from lupus patients.
Topics: 1-Deoxynojirimycin; Adult; Aged; Antigens, CD; B-Lymphocytes; CD4-Positive T-Lymphocytes; Female; Flow Cytometry; G(M1) Ganglioside; Gene Expression Regulation; Glycosphingolipids; Homeostasis; Humans; Lactosylceramides; Leukocytes, Mononuclear; Liver X Receptors; Lupus Erythematosus, Systemic; Lymphocyte Activation; Male; Membrane Microdomains; Middle Aged; Orphan Nuclear Receptors; Signal Transduction; Time Factors; Trihexosylceramides | 2014 |
Molecular basis of 1-deoxygalactonojirimycin arylthiourea binding to human α-galactosidase a: pharmacological chaperoning efficacy on Fabry disease mutants.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Animals; Autophagy; Chlorocebus aethiops; COS Cells; Crystallography, X-Ray; Enzyme Stability; Fabry Disease; Fibroblasts; Humans; Molecular Docking Simulation; Mutation; Protein Transport; Thiourea; Trihexosylceramides | 2014 |
Reduction of podocyte globotriaosylceramide content in adult male patients with Fabry disease with amenable
Topics: 1-Deoxynojirimycin; Adult; alpha-Galactosidase; Enzyme Inhibitors; Fabry Disease; Humans; Male; Middle Aged; Podocytes; Treatment Outcome; Trihexosylceramides | 2017 |
Glucosylceramide synthase inhibition with lucerastat lowers globotriaosylceramide and lysosome staining in cultured fibroblasts from Fabry patients with different mutation types.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Cell Line; Fabry Disease; Female; Fibroblasts; Genotype; Glucosyltransferases; Humans; Kidney; Lysosomes; Male; Mutation; Trihexosylceramides | 2018 |
Mutation-specific Fabry disease patient-derived cell model to evaluate the amenability to chaperone therapy.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Cell- and Tissue-Based Therapy; Enzyme Replacement Therapy; Fabry Disease; Gene Editing; HEK293 Cells; Humans; Molecular Chaperones; Precision Medicine; Trihexosylceramides | 2019 |
[The Fabry nephropathy: new insight in diagnosis, monitoring and treatment].
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Disease Progression; Enzyme Replacement Therapy; Fabry Disease; Female; Glomerulosclerosis, Focal Segmental; Glycolipids; Heterozygote; Humans; Isoenzymes; Kidney Diseases; Male; Oxidative Stress; Podocytes; Recombinant Proteins; Sex Factors; Sphingolipids; Trihexosylceramides | 2019 |