دانلود مقالات ISI درباره N-Butyldeoxynojirimycin + ترجمه فارسی

Excessive interferon-α signaling in autoimmunity alters glycosphingolipid processing in B cells

Keywords: Autoimmunity; B cell; iNKT cell; Interferon-α; Glycosphingolipid; GSL metabolism; IFN; interferon; TNF; tumour necrosis factor; IL; interleukin; iNKT; invariant natural killer T; GSL; glycosphingolipid; Cer; ceramide; LacCer; lactosylceramide; GCS; gluco

دانلود رایگان متن کامل مقاله ISI 10 صفحه سال انتشار : 2018 سفارش ترجمه

N-butyldeoxynojirimycin delays motor deficits, cerebellar microgliosis, and Purkinje cell loss in a mouse model of mucolipidosis type IV

Keywords: 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

دانلود رایگان متن کامل مقاله ISI 14 صفحه سال انتشار : 2017 سفارش ترجمه

Research ArticleRescue of defective ATP8B1 trafficking by CFTR correctors as a therapeutic strategy for familial intrahepatic cholestasis

Keywords: CFTR; cystic fibrosis transmembrane conductance regulator; 4-PBA; 4-phenyl butyric acid; SAHA; suberoylanilide hydroxamic acid; NB-DNJ; N-butyldeoxynojirimycin; BRIC; benign recurrent intrahepatic cholestasis; PFIC; progressive familial intrahepatic chole

دانلود رایگان متن کامل مقاله ISI 9 صفحه سال انتشار : 2016 سفارش ترجمه

Lack of enzyme activity in GBA2 mutants associated with hereditary spastic paraplegia/cerebellar ataxia (SPG46)

Keywords: 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

دانلود رایگان متن کامل مقاله ISI 6 صفحه سال انتشار : 2015 سفارش ترجمه

Synthetic ceramide analogues increase amyloid-β 42 production by modulating γ-secretase activity

Keywords: AD; Alzheimer disease; FAD; familial AD; Aβ; amyloid-β peptide; APP; amyloid precursor protein; BACE1; β-site APP cleaving enzyme 1; PDMP; dl-threo-1-phenyl-2-decanoylamino-3-morpholino-1-propanol; DMAPP; (1S,2R-d-erythro-2-N-myristoylamino)-1-phenyl-1

دانلود رایگان متن کامل مقاله ISI 6 صفحه سال انتشار : 2015 سفارش ترجمه

Selective chaperone effect of aminocyclitol derivatives on G202R and other mutant glucocerebrosidases causing Gaucher disease

Keywords: BBB; blood-brain barrier; CBE; conduritol β-epoxide; Cer; ceramide; D-MEM; Dulbeco Eagle's minimal essential medium; ERT; enzyme replacement therapy; GBA1; lysosomal glucocerebrosidase; GBA2; non-lysosomal glucocerebrosidase; GlcCer; glucosylceramide;

دانلود رایگان متن کامل مقاله ISI 10 صفحه سال انتشار : 2014 سفارش ترجمه

The identification and molecular characterization of the first archaeal bifunctional exo-β-glucosidase/N-acetyl-β-glucosaminidase demonstrate that family GH116 is made of three functionally distinct subfamilies

Keywords: CAZy; carbohydrate active enzyme classification; X-GlcNAc; 5-bromo-4-chloro-indolyl-N-acetyl-β-d-glucosaminide; 4NP-GlcNAc; 4-nitropheny-N-acetyl-β-d-glucosaminide; MS; mass spectrometry; 4Np-Glc; 4Np-β-glucopyranoside; Glc; glucose; GlcNAc; N-acetylgl

دانلود رایگان متن کامل مقاله ISI 11 صفحه سال انتشار : 2014 سفارش ترجمه

Recommendations for the diagnosis and management of Niemann-Pick disease type C: An update

Keywords: ACE; Addenbrooke's Cognitive Examination; ASM; acid sphingomyelinase; ALAT; alanine aminotransferase; ASAT; aspartate aminotransferase; BARS; Brief Ataxia Rating Scale; BPRS; Brief Psychiatric Rating Scale; BSID; Bayley Scales for Infant Development; cDNA

دانلود رایگان متن کامل مقاله ISI 15 صفحه سال انتشار : 2012 سفارش ترجمه

Glycosphingolipid depletion in PC12 cells using iminosugars protects neuronal membranes from anti-ganglioside antibody mediated injury

Keywords: Iminosugars; Glucosylceramide synthase; N-butyldeoxynojirimycin; Ganglioside; Antibody; Neuropathy

دانلود رایگان متن کامل مقاله ISI 6 صفحه سال انتشار : 2008 سفارش ترجمه

Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis

Keywords: GM1 gangliosidosis; Lysosomal storage disease; Glycosphingolipid; GM1; GA1; Substrate reduction therapy; Miglustat; Imino sugar; N-Butyldeoxynojirimycin; N-Butyldeoxygalactonojirimycin;

دانلود رایگان متن کامل مقاله ISI 8 صفحه سال انتشار : 2008 سفارش ترجمه

Selective action of the iminosugar isofagomine, a pharmacological chaperone for mutant forms of acid-β-glucosidase

Keywords: GlcCerase, acid-β-glucosidase; IFG, isofagomine; NB-DNJ, N-butyldeoxynojirimycinIsofagomine; N-Butyldeoxynojirimycin; Gaucher; Acid-β-glucosidase; Disaccharidase; α-Glucosidase II

دانلود رایگان متن کامل مقاله ISI 8 صفحه سال انتشار : 2007 سفارش ترجمه