Keywords: مگولاستا; NPC; Early infantile; Miglustat; Lung involvement;
مقالات ISI مگولاستا (ترجمه نشده)
مقالات زیر هنوز به فارسی ترجمه نشده اند.
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در صورتی که به ترجمه آماده هر یک از مقالات زیر نیاز داشته باشید، می توانید سفارش دهید تا مترجمان با تجربه این مجموعه در اسرع وقت آن را برای شما ترجمه نمایند.
Keywords: مگولاستا; Hemolytic uremic syndrome; Shiga toxin type 2; Miglustat; Human glomerular endothelial cells; Immortalized human proximal tubule epithelial cellsHUS, Hemolytic Uremic Syndrome; Stx2, Shiga toxin type 2; STEC, Shiga toxin (Stx)-producing Escherichia coli;
Keywords: مگولاستا; Niemann–Pick C; Miglustat; Psychosis; Cerebral atrophy
Keywords: مگولاستا; NPC; Niemann-Pick disease type C; CS; composite score; BS; body surface; LE/L; late endosomes/lysosomes; ER; endoplasmic reticulum; Niemann-Pick type C; Miglustat; Disability scale; Mean annual change; Dysphagia; Epilepsy;
Molecular and biochemical biomarkers for diagnosis and therapy monitorization of Niemann-Pick type C patients
Keywords: مگولاستا; NP-C; Niemann-Pick type C; LSD; lysosomal storage disorder; CNS; central nervous system; 7-KC; 7-ketocholesterol; GC/MS; gas chromatography/mass spectrometry; LC/MS-MS; liquid chromatography/tandem mass spectrometry; LDL; low density lipoprotein; LE; late
Twelve years of experience with miglustat in the treatment of type 1 Gaucher disease: The Spanish ZAGAL project
Keywords: مگولاستا; BUA; broadband ultrasound attenuation; BMD; bone mineral density; CCL18/PARC; chemokine (C-C motif) ligand 18/pulmonary and activation-regulated chemokine; CHITO; chitotriosidase; ERT; enzyme replacement therapy; DXA; dual X-ray absorptiometry; EMG; elect
Infantile gangliosidoses: Mapping a timeline of clinical changes
Keywords: مگولاستا; Disaccharidase; Gangliosidosis; Ganglioside; Ketogenic diet; Substrate; Miglustat;
One pot oxidative dehydration - oxidation of polyhydroxyhexanal oxime to polyhydroxy oxohexanenitrile: A versatile methodology for the facile access of azasugar alkaloids
Keywords: مگولاستا; Deoxynojirimycin; Miglitol; Miglustat; Alcohol oxidation & oxime dehydration; Cascade reaction; Reductive aminative cyclization;
Urinary excretion and metabolism of miglustat and valproate in patients with Niemann-Pick type C1 disease: One- and two-dimensional solution-state 1H NMR studies
Keywords: مگولاستا; Miglustat (CID 51634); Valproate (CID 3121); 1-O-Valproyl-β-glucuronide (53477719); NP-C; Niemann-Pick type C disease; GSL; glycosphingolipid; MGS; miglustat; UD; puridine diphosphate; UDPGA; uridine 5'-diphosphoglucuronic acid trisodium salt; NP-C d
Lack of enzyme activity in GBA2 mutants associated with hereditary spastic paraplegia/cerebellar ataxia (SPG46)
Keywords: مگولاستا; Glucosylceramide; Spastic paraplegia; Cerebellar ataxia; Miglustat; GBA2; Enzyme activity; SPG46; Cer; ceramide; GBA; glucocerebrosidase; GBA2; β-glucosidase 2; Glc; glucose; GlcCer; glucosylceramide; NB-DGJ; N-butyldeoxygalactonojirimycin; NB-DNJ; N-but
A case report of ‘variant’ biochemical phenotype of Niemann-Pick C disease and a discussion of therapeutic options
Keywords: مگولاستا; Niemann-Pick type C; heterozygote mutation; miglustatchoroba Niemanna-Picka typu C; mutacja złożona; miglustat
Miglustat therapy in type 1 Gaucher disease: Clinical and safety outcomes in a multicenter retrospective cohort study
Keywords: مگولاستا; CI, confidence interval; CT, computed tomography; ERT, enzyme replacement therapy; GD/GD1, Gaucher disease/Gaucher disease type 1; MRI, magnetic resonance imaging; SD, standard deviation; SRT, substrate reduction therapyGaucher disease; Miglustat; Outcome
Unexpected cure from cutaneous leukocytoclastic vasculitis in a patient treated with N-butyldeoxynojirimycin (miglustat) for Gaucher disease
Keywords: مگولاستا; Gaucher disease; Inflammation; Leukocytoclastic vasculitis; Miglustat
A randomized placebo-controlled trial of miglustat in cystic fibrosis based on nasal potential difference
Keywords: مگولاستا; Miglustat; Cystic fibrosis; F508del; Efficacy; Tolerability; Pharmacokinetics
Validated LC–MS/MS method for the quantitative determination of the glucosylceramide synthase inhibitor miglustat in mouse plasma and human plasma and its application to a pharmacokinetic study
Keywords: مگولاستا; Miglustat; N-(n-nonyl)deoxynojirimycin; Assay validation; Pharmacokinetics; Accurate mass
Gaucher disease due to saposin C deficiency, previously described as non-neuronopathic form - No positive effects after 2-years of miglustat therapy
Keywords: مگولاستا; Gaucher disease variant; Saposin C; Substrate deprivation therapy; Miglustat;
Treatment of cataplexy in Niemann–Pick disease type C with the use of miglustat
Keywords: مگولاستا; Cataplexy; Niemann–Pick type C; Miglustat; Children
La maladie de Niemann-Pick de type C
Keywords: مگولاستا; Niemann Pick C; Test à la Filipine; Miglustat; Ophtalmoplégie supranucléaire; Niemann-Pick C; Filipine test; Miglustat; Supranuclear ophthalmoplegia;
Eye movement and diffusion tensor imaging analysis of treatment effects in a Niemann-Pick Type C patient
Keywords: مگولاستا; Niemann-Pick Type C; Miglustat; DTI; Fractional anisotropy;
Changes in the atherogenic profile of patients with type 1 Gaucher disease after miglustat therapy
Keywords: مگولاستا; Atherogenic; Miglustat; Substrate reduction therapy; Coronary artery disease (CAD); Lipids; Lipoprotein;
Miglustat in adult and juvenile patients with Niemann–Pick disease type C: Long-term data from a clinical trial
Keywords: مگولاستا; Niemann–Pick type C; Miglustat; Long-term; Efficacy; Safety
Clinical experience with miglustat therapy in pediatric patients with Niemann–Pick disease type C: A case series
Keywords: مگولاستا; Niemann–Pick disease type C (NP-C); Miglustat; PET; Disability; Chitotriosidase, CCL18
24 month-treatment with miglustat of three patients with Niemann-Pick disease type C: Follow up using brain spectroscopy
Keywords: مگولاستا; Niemann-Pick C; Miglustat; Zavesca; Magnetic resonance spectroscopy; Inherited metabolic diseases; Inborn errors of metabolism; Substrate reduction therapy
Rapid quantification of miglustat in human plasma and cerebrospinal fluid by liquid chromatography coupled with tandem mass spectrometry
Keywords: مگولاستا; Miglustat; OGT 918; Deoxynojirimycin; Mass spectrometry; Liquid chromatography
Miglustat in patients with Niemann-Pick disease Type C (NP-C): A multicenter observational retrospective cohort study
Keywords: مگولاستا; Niemann-Pick disease Type C; Miglustat; Observational; Cohort; Progression; Disability
Pharmacokinetics, safety and tolerability of miglustat in the treatment of pediatric patients with GM2 gangliosidosis
Keywords: مگولاستا; GM2 gangliosidosis; Miglustat; Pharmacokinetics; Safety; Sandhoff disease; Substrate reduction therapy; Tay–Sachs disease
Beneficial effects of anti-inflammatory therapy in a mouse model of Niemann-Pick disease type C1
Keywords: مگولاستا; Niemann-Pick disease type C; CNS inflammation; Non-steroidal anti-inflammatory drugs; NSAIDs; Miglustat
Substrate reduction therapy in juvenile GM2 gangliosidosis
Keywords: مگولاستا; Juvenile GM2 gangliosidosis; Miglustat; Sandhoff disease; Substrate reduction therapy; Tay–Sachs disease
Étude rétrospective sur dix ans des patients atteints de maladie de Gaucher au CHU de Rennes
Keywords: مگولاستا; Maladie de Gaucher; Gammapathie monoclonale; Syndrome parkinsonien; Imiglucérase; MiglustatGaucher's disease; Monoclonal gammopathy; Parkinsonism; Imiglucerase; Miglustat
Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis
Keywords: مگولاستا; GM1 gangliosidosis; Lysosomal storage disease; Glycosphingolipid; GM1; GA1; Substrate reduction therapy; Miglustat; Imino sugar; N-Butyldeoxynojirimycin; N-Butyldeoxygalactonojirimycin;
Calcium homeostasis is abnormal in cystic fibrosis airway epithelial cells but is normalized after rescue of F508del-CFTR
Keywords: مگولاستا; Cystic fibrosis; Calcium signalling; Pharmacology; F508del-CFTR; Trafficking; Miglustat;
Effective treatment of an elderly patient with Gaucher's disease and Parkinsonism: A case report of 24 months’ oral substrate reduction therapy with miglustat
Keywords: مگولاستا; Gaucher's disease; Parkinson's disease; Miglustat; Substrate reduction therapy
Miglustat (NB-DNJ) works as a chaperone for mutated acid β-glucosidase in cells transfected with several Gaucher disease mutations
Keywords: مگولاستا; Gaucher disease; Acid β-glucosidase; N-butyl-deoxynojirimycin; Miglustat; Glucosylceramide; Chemical chaperone; Docking;
An open-label, noncomparative study of miglustat in type I Gaucher disease: Efficacy and tolerability over 24 months of treatment
Keywords: مگولاستا; miglustat; Gaucher disease; substrate reduction therapy; liver; spleen; platelets;