Keywords: درمان با سوبسترا; Gaucher; Imiglucerase; Substrate reduction therapy;
مقالات ISI درمان با سوبسترا (ترجمه نشده)
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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: درمان با سوبسترا; Gaucher disease type 1; Acid β-glucosidase deficiency; Eliglustat; Substrate reduction therapy; Safety; Adverse events;
Keywords: درمان با سوبسترا; Glucocerebrosidase deficiency; Lysosomal storage disease; Resting energy expenditure; Insulin resistance; High-density lipoprotein cholesterol; Hepatocellular carcinoma; Gaucher disease; ApoAI; apolipoprotein AI; ApoB; apolipoprotein B; ApoE; apolipoprote
Keywords: درمان با سوبسترا; Gaucher disease; Hyperferritinemia; Cytokines; TNF-α; Enzyme replacement therapy; Substrate reduction therapy;
Keywords: درمان با سوبسترا; Enzyme replacement therapy; Gaucher; macrophage; substrate reduction therapy;
Keywords: درمان با سوبسترا; BMD; bone mineral density; ECG; electrocardiogram; ERT; enzyme replacement therapy; ICGG; International Collaborative Gaucher Group; Type 1 Gaucher disease; Eliglustat; Substrate reduction therapy; Enzyme replacement/augmentation therapy; Drug metabolism;
Keywords: درمان با سوبسترا; AAV; adeno-associated viruses; AE; adverse event; BBB; blood-brain barrier; CLN2; ceroid lipofuscinosis neuronal type 2; CNS; central nervous system; CSF; cerebrospinal fluid; DQ; developmental quotient; ERT; enzyme replacement therapy; g7; 7-aminoacid gl
Keywords: درمان با سوبسترا; ANOVA; one-way analysis of variance; BMD; bone mineral density; BMI; body mass index; CCL18; chemokine (C-C motif) ligand 18; EC; Enzyme commission number; ERT; enzyme replacement therapy; GBA; glucocerebrosidase; GD; Gaucher disease; DXA; dual energy X-r
Keywords: درمان با سوبسترا; Lysosomal storage disease; Cardiomyopathy; HEXB; Substrate reduction therapy; Ketogenic diet
Enzyme replacement therapy reverses B lymphocyte and dendritic cell dysregulations in patients with Gaucher Disease
Keywords: درمان با سوبسترا; ACE; angiotensin conversion enzyme; CBC; complete blood count; CHITO; chitotriosidase; DC; dendritic cells; ERT; enzyme replacement therapy; GCase; Glucocerebrosidase; HC; healthy control; Hgb; hemoglobin; Ig; Immunoglobulin; MGUS; monoclonal gammopathy o
Once- versus twice-daily dosing of eliglustat in adults with Gaucher disease type 1: The Phase 3, randomized, double-blind EDGE trial
Keywords: درمان با سوبسترا; Gaucher disease type 1; Eliglustat; Substrate reduction therapy; Dosing study; Clinical trial;
Generation of osteoclasts from type 1 Gaucher patients and correlation with clinical and genetic features of disease
Keywords: درمان با سوبسترا; GD1; Gaucher disease type 1; AVN; avascular necrosis; BMD; bone mineral density; GD; Gaucher disease; ERT; enzyme replacement therapy; SRT; substrate reduction therapy; DEXA; dual energy X-ray absorptiometry; CCL18; chemokine (C-C motif) ligand 18; MIP1-Î
N-butyldeoxynojirimycin delays motor deficits, cerebellar microgliosis, and Purkinje cell loss in a mouse model of mucolipidosis type IV
Keywords: درمان با سوبسترا; NB-DNJ; N-butyldeoxynojirimycin; GCS; glucosylceramide synthase; GSL; glycosphingolipid; CMH; ceramide monohexoside; CDH; ceramide dihexoside; GBA2; glucocerebrosidase; NPC; Niemann-Pick type C; SRT; substrate reduction therapy; Mucolipidosis type IV; Mig
Ghrelin, leptin and adiponectin levels in Gaucher disease type I patients on enzyme replacement therapy
Keywords: درمان با سوبسترا; Gaucher disease type I; Adiponectin; Ghrelin; Leptin; Insulin; BMI; body mass index; ERT; enzyme replacement therapy; GD; Gaucher disease; HOMA-IR; homeostatic model assessment of insulin resistance; SRT; substrate reduction therapy; T2DM; type 2 diabetes
Iminosugar-based inhibitors of glucosylceramide synthase prolong survival but paradoxically increase brain glucosylceramide levels in Niemann–Pick C mice
Keywords: درمان با سوبسترا; Niemann–Pick type C disease; Substrate reduction therapy; Lysosomal storage disease; Glucosylceramide; GBA2 gene; Glucosylceramide synthase
Changes in the atherogenic profile of patients with type 1 Gaucher disease after miglustat therapy
Keywords: درمان با سوبسترا; Atherogenic; Miglustat; Substrate reduction therapy; Coronary artery disease (CAD); Lipids; Lipoprotein;
24 month-treatment with miglustat of three patients with Niemann-Pick disease type C: Follow up using brain spectroscopy
Keywords: درمان با سوبسترا; Niemann-Pick C; Miglustat; Zavesca; Magnetic resonance spectroscopy; Inherited metabolic diseases; Inborn errors of metabolism; Substrate reduction therapy
Pharmacokinetics, safety and tolerability of miglustat in the treatment of pediatric patients with GM2 gangliosidosis
Keywords: درمان با سوبسترا; GM2 gangliosidosis; Miglustat; Pharmacokinetics; Safety; Sandhoff disease; Substrate reduction therapy; Tay–Sachs disease
Genistein reduces lysosomal storage in peripheral tissues of mucopolysaccharide IIIB mice
Keywords: درمان با سوبسترا; Mucopolysaccharidosis; Genistein; Substrate reduction therapy; Glycosaminoglycan; Sanfilippo syndrome IIIB; Naglu; Lysosomal storage
Substrate reduction therapy in juvenile GM2 gangliosidosis
Keywords: درمان با سوبسترا; Juvenile GM2 gangliosidosis; Miglustat; Sandhoff disease; Substrate reduction therapy; Tay–Sachs disease
A 25-year longitudinal analysis of treatment efficacy in inborn errors of metabolism
Keywords: درمان با سوبسترا; Inborn errors of metabolism; Networks; The Internet; Protein restriction; Enzyme replacement therapy; Substrate reduction therapy; Cell transplantation; Gene therapy
Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis
Keywords: درمان با سوبسترا; GM1 gangliosidosis; Lysosomal storage disease; Glycosphingolipid; GM1; GA1; Substrate reduction therapy; Miglustat; Imino sugar; N-Butyldeoxynojirimycin; N-Butyldeoxygalactonojirimycin;
Therapy for Gaucher disease: Don’t stop thinking about tomorrow
Keywords: درمان با سوبسترا; Gaucher disease; Enzyme replacement therapy; Lysosomal storage diseases; Chaperone therapy; Gene therapy; Substrate reduction therapy
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: درمان با سوبسترا; Gaucher's disease; Parkinson's disease; Miglustat; Substrate reduction therapy
An open-label, noncomparative study of miglustat in type I Gaucher disease: Efficacy and tolerability over 24 months of treatment
Keywords: درمان با سوبسترا; miglustat; Gaucher disease; substrate reduction therapy; liver; spleen; platelets;