دانلود مقالات ISI درباره نوع Niemann Pick نوع C + ترجمه فارسی

تأثیر تجزیه بیومارکرها در تشخیص بیماری نیمن-پیچ سی و کمبود اسکینگوینیناز اسید

The impact of biomarkers analysis in the diagnosis of Niemann-Pick C disease and acid sphingomyelinase deficiency

Keywords: نوع Niemann Pick نوع C; Oxysterols; Triol; 7-Ketocholesterol; Lysosphingomyelin; Lysosphingomyelin-509; Niemenn Pick disease type C; Acid sphingomyelinase deficiency; NP-C; Niemann-Pick type C; ASMD; Acid Sphingomielinase Deficiency; Triol; Cholestan-3β,5α,6β-triol; 7-KC; 7-k

دانلود رایگان متن کامل مقاله ISI 8 صفحه سال انتشار : 2018 سفارش ترجمه

سنتز و مشخصه یک ردمامین با عنوان خبرنگار کلسترول

Synthesis and characterization of a novel rhodamine labeled cholesterol reporter

Keywords: نوع Niemann Pick نوع C; ACAT; acyl-coenzyme A:cholesterol acyl transferase; DChol; 6-dansyl-cholestanol; RChol; 6-rhodamine-cholestanol; BChol; bodipy cholesterol (= TopFluo Chosterol); BODIPY; boron dipyrro methane; BSA; bovine serum albumin; DOPC; 1,2-dioleoyl-sn-glycero-3-ph

دانلود رایگان متن کامل مقاله ISI 50 صفحه سال انتشار : 2017 سفارش ترجمه

تجزیه و تحلیل نقش هدف سیگنالینگ رپامایسین در متابولیسم اسپینگولایپید

Unraveling the role of the Target of Rapamycin signaling in sphingolipid metabolism

Keywords: نوع Niemann Pick نوع C; AbA; Aureobasidin A; ABC; ATP-binding cassette; AMPK; AMP-activated protein kinase; ATG; Autophagy-related; ATP; Adenosine triphosphate; C1P; Ceramide-1-phosphate; CERS; Ceramide synthase; CK2; Casein kinase II; CLS; Chronological lifespan; CMA; Chaperone

دانلود رایگان متن کامل مقاله ISI 25 صفحه سال انتشار : 2016 سفارش ترجمه

Molecular and biochemical biomarkers for diagnosis and therapy monitorization of Niemann-Pick type C patients

Keywords: نوع Niemann Pick نوع C; 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

دانلود رایگان متن کامل مقاله ISI 6 صفحه سال انتشار : 2018 سفارش ترجمه

Role of 6-O-α-maltosyl-β-cyclodextrin in lysosomal cholesterol deprivation in Npc1-deficient Chinese hamster ovary cells

Keywords: نوع Niemann Pick نوع C; 6-O-α-maltosyl-β-cyclodextrin (Mal-βCD); Npc1-deficient cells; Lysosomal unesterified cholesterol; Cholesterol/efflux; Endocytosis; Liquid chromatography-mass spectrometry; βCD; β-cyclodextrin; CHO; Chinese hamster ovary; DAOS; (hydroxy-3-sulfopropyl

دانلود رایگان متن کامل مقاله ISI 8 صفحه سال انتشار : 2018 سفارش ترجمه

Novel homozygous GBA2 mutation in a patient with complicated spastic paraplegia

Keywords: نوع Niemann Pick نوع C; AD; autosomal dominant; AOA1; ataxia with oculomotor apraxia type 1; AR; autosomal recessive; ARCA; autosomal recessive cerebellar ataxia; ATM; ataxia talengiectasia; FA; Friedreich ataxia; GBA2; b-glucosidase 2; HSPs; hereditary spastic paraplegias; NGS;

دانلود رایگان متن کامل مقاله ISI 11 صفحه سال انتشار : 2018 سفارش ترجمه

Long-Term Treatment of Niemann-Pick Type C1 Disease With Intrathecal 2-Hydroxypropyl-β-Cyclodextrin

Keywords: نوع Niemann Pick نوع C; Niemann-Pick type C; Lysosomal storage disorder; Neurodegenerative disease; Cholesterol; Cyclodextrin;

دانلود رایگان متن کامل مقاله ISI 49 صفحه سال انتشار : 2018 سفارش ترجمه

A saposin deficiency model in Drosophila: Lysosomal storage, progressive neurodegeneration and sensory physiological decline

Keywords: نوع Niemann Pick نوع C; PSAP; prosaposin; ERG; electroretinograms; LSD; lysosomal storage disease; NPC; Niemann-Pick type C; GFP; green fluorescent protein; RT-PCR; reverse transcription PCR; TEM; transmission electron microscopy; MVB; multivesicular body; MLB; multilamellar bod

دانلود رایگان متن کامل مقاله ISI 11 صفحه سال انتشار : 2017 سفارش ترجمه

Annexin A6 in the liver: From the endocytic compartment to cellular physiology

Keywords: نوع Niemann Pick نوع C; ABC; adenosine triphosphate binding cassette transporters; AEE; apical early endosomes; AnxA6; annexin group A; ARE; apical recycling endosomes; ASGP; asialoglycoprotein receptor; BFA; Brefeldin A; BEE; basolateral early endosomes; CaM; calmodulin; Cav-1;

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

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

Keywords: نوع Niemann Pick نوع C; 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

In vitro evaluation of 2-hydroxyalkylated β-cyclodextrins as potential therapeutic agents for Niemann-Pick Type C disease

Keywords: نوع Niemann Pick نوع C; Niemann-Pick Type C; Hydroxypropyl-β-cyclodextrin; Lysosomal storage disease; Cholesterol;

دانلود رایگان متن کامل مقاله ISI 6 صفحه سال انتشار : 2016 سفارش ترجمه

MicroRNAs in brain cholesterol metabolism and their implications for Alzheimer's disease

Keywords: نوع Niemann Pick نوع C; AAV; adeno associated virus; ABC; ATP-binding cassette; ABCA1; ABC transporter A1; AD; Alzheimer's disease; APOA-I; apolipoprotein A1; APOE; apolipoprotein E; APP; amyloid β precursor protein; BBB; blood-brain barrier; CE; cholesteryl ester; CETP; chol

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

Astrocytes and lysosomal storage diseases

Keywords: نوع Niemann Pick نوع C; AD; Alzheimer's disease; DAMP; danger-associated molecular pattern; GBA; glucocerebrosidase; GFAP; glial fibrillary acidic protein; GLD; globoid cell leukodystrophy; INCL; infantile neuronal ceroid lipofuscinosis; JNCL; juvenile neuronal ceroid lipofusc

دانلود رایگان متن کامل مقاله ISI 12 صفحه سال انتشار : 2016 سفارش ترجمه

How to reduce the accumulation of autophagic vacuoles in NPC1-deficient neurons: A comparison of two pharmacological strategies

Keywords: نوع Niemann Pick نوع C; Neurons; Niemann-Pick type C disease; Autophagy; Cyclodextrin; PI3K-inhibitor; CD; cyclodextrin; pCD; 2-hydroxy-propyl-β-cyclodextrin; LC3; microtubule-associated protein 1 light chain 3; LC3-I; phosphatidylethanolamine-deconjugated LC3; LC3-II; phosphat

دانلود رایگان متن کامل مقاله ISI 8 صفحه سال انتشار : 2015 سفارش ترجمه

GGA1 overexpression attenuates amyloidogenic processing of the amyloid precursor protein in Niemann-Pick type C cells

Keywords: نوع Niemann Pick نوع C; Aβ; amyloid beta peptide; AD; Alzheimer's disease; ADAM; A disintegrin and metalloproteinase; APOE; apolipoprotein E; APP; amyloid precursor protein; BACE1; beta-secretase; CHO; Chinese hamster ovary; CTF; C-terminal fragment; DN; dominant negative; FA

دانلود رایگان متن کامل مقاله ISI 6 صفحه سال انتشار : 2014 سفارش ترجمه

A case report of ‘variant’ biochemical phenotype of Niemann-Pick C disease and a discussion of therapeutic options

Keywords: نوع Niemann Pick نوع C; Niemann-Pick type C; heterozygote mutation; miglustatchoroba Niemanna-Picka typu C; mutacja złożona; miglustat

دانلود رایگان متن کامل مقاله ISI 4 صفحه سال انتشار : 2013 سفارش ترجمه

Cerebellar volume correlates with saccadic gain and ataxia in adult Niemann-Pick type C

Keywords: نوع Niemann Pick نوع C; DTI, Diffusion tensor imaging; MRI, Magnetic resonance imaging; NAA, N-acetyl aspartate; NPC, Niemann-Pick disease type C; SPGR, Spoiled-gradient recalled echoCerebellum; Niemann-Pick type C; White matter; Lysosomal; Ataxia; Gaze palsy

دانلود رایگان متن کامل مقاله ISI 5 صفحه سال انتشار : 2013 سفارش ترجمه

The Cholesterol Derivative 27-Hydroxycholesterol Reduces Steatohepatitis in Mice

Keywords: نوع Niemann Pick نوع C; Metabolic Syndrome; Fatty Liver; NAFLD; Mouse Model; 27HC; 27-hydroxycholesterol (25(R)26 hydroxycholesterol); ALT; alanine aminotransferases; COL1A1; collagenase 1A1; CTX; cerebrotendinous xanthomatosis; HFC; high-fat high cholesterol; IL; interleukin; K

دانلود رایگان متن کامل مقاله ISI 13 صفحه سال انتشار : 2013 سفارش ترجمه

Uptake of a fluorescent methyl-β-cyclodextrin via clathrin-dependent endocytosis

Keywords: نوع Niemann Pick نوع C; CD(s); cyclodextrin(s); CHC; clathrin heavy chain; CPZ; chlorpromazine; DMEM; Dulbecco's modified Eagle's medium; DPBS; Dulbecco's Phosphate Buffered Saline; EPR; electron paramagnetic resonance; FCS; fetal calf serum; FMβCD; randomly methylated 6I-monod

دانلود رایگان متن کامل مقاله ISI 7 صفحه سال انتشار : 2012 سفارش ترجمه

Oxidative stress activates the c-Abl/p73 proapoptotic pathway in Niemann-Pick type C neurons

Keywords: نوع Niemann Pick نوع C; Niemann-Pick type C; c-Abl/p73; Oxidative stress; Apoptosis; Neurodegeneration

دانلود رایگان متن کامل مقاله ISI 10 صفحه سال انتشار : 2011 سفارش ترجمه

Defective cholesterol trafficking in Niemann-Pick C-deficient cells

Keywords: نوع Niemann Pick نوع C; ACAT; acyl-CoA:cholesterol acyltransferase; ALLO; allopregnanolone; CD; cyclodextrin; CE; cholesterol esters; CNS; central nervous system; ER; endoplasmic reticulum; GSL; glycosphingolipid; LDL; low density lipoprotein; LE/L; late endosomes/lysosomes; NPC

دانلود رایگان متن کامل مقاله ISI 9 صفحه سال انتشار : 2010 سفارش ترجمه

Intracellular sterol dynamics

Keywords: نوع Niemann Pick نوع C; PM; plasma membrane; ER; endoplasmic reticulum; ERC; endosomal recycling compartment; LE; late endosome; LY; lysosome; LSO; LE/LY-like storage organelle; PC; phosphatidylcholine; SM; sphingomyelin; PS; phosphatidylserine; PE; phosphatidylethanolamine; DO;

دانلود رایگان متن کامل مقاله ISI 10 صفحه سال انتشار : 2009 سفارش ترجمه

Pharmacological regulation of low density lipoprotein receptor expression: Current status and future developments

Keywords: نوع Niemann Pick نوع C; LDL; low density lipoprotein; HDL; high density lipoprotein; TG; triglycerides; apoB; apolipoprotein B; SREBP; sterol responsive element binding protein; SCAP; SREBP cleavage activating protein; Insig; insulin-induced gene; NPC; Niemann-Pick Type C; OM; o

دانلود رایگان متن کامل مقاله ISI 10 صفحه سال انتشار : 2006 سفارش ترجمه