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