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35506864 | Lactose-Appended Hydroxypropyl-β-Cyclodextrin Lowers Cholesterol Accumulation and Alleviates Motor Dysfunction in Niemann-Pick Type C Disease Model Mice. | 2022 May 16 |
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33256498 | Gene Therapy in a Mouse Model of Niemann-Pick Disease Type C1. | 2021 Jun |
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33345999 | Lack of Annexin A6 Exacerbates Liver Dysfunction and Reduces Lifespan of Niemann-Pick Type C Protein-Deficient Mice. | 2021 Mar |
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33503462 | Beneficial effects of primidone in Niemann-Pick disease type C (NPC)-model cells and mice: Reduction of unesterified cholesterol levels in cells and extension of lifespan in mice. | 2021 Apr 5 |
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33687199 | Visualizing Cholesterol in the Brain by On-Tissue Derivatization and Quantitative Mass Spectrometry Imaging. | 2021 Mar 23 |
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33772019 | Loss of bone morphogenetic protein-binding endothelial regulator causes insulin resistance. | 2021 Mar 26 |
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33802460 | GCase and LIMP2 Abnormalities in the Liver of Niemann Pick Type C Mice. | 2021 Mar 3 |
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| 8 |
34023384 | Enrichment of NPC1-deficient cells with the lipid LBPA stimulates autophagy, improves lysosomal function, and reduces cholesterol storage. | 2021 Jul |
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| 9 |
34175669 | Acid ceramidase improves mitochondrial function and oxidative stress in Niemann-Pick type C disease by repressing STARD1 expression and mitochondrial cholesterol accumulation. | 2021 Sep |
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| 10 |
34364974 | Glial contribution to cyclodextrin-mediated reversal of cholesterol accumulation in murine NPC1-deficient neurons in vivo. | 2021 Oct |
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| 11 |
34948052 | Impaired Retromer Function in Niemann-Pick Type C Disease Is Dependent on Intracellular Cholesterol Accumulation. | 2021 Dec 9 |
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| 12 |
35095535 | The Effects of Combined Therapy With Metformin and Hydroxypropyl-β-Cyclodextrin in a Mouse Model of Niemann-Pick Disease Type C1. | 2021 |
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| 13 |
30118401 | Enhanced activity of superoxide dismutase is a common response to dietary and genetically induced increased cholesterol levels. | 2020 May |
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| 14 |
31312996 | Ontogenesis and Modulation of Intestinal Unesterified Cholesterol Sequestration in a Mouse Model of Niemann-Pick C1 Disease. | 2020 Jan |
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| 15 |
31599924 | Stimulation of mGluR1/5 Improves Defective Internalization of AMPA Receptors in NPC1 Mutant Mouse. | 2020 Mar 14 |
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31862414 | Decreased membrane cholesterol in liver mitochondria of the point mutation mouse model of juvenile Niemann-Pick C1, Npc1nmf164. | 2020 Mar |
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| 17 |
32291331 | Dietary plant stanol ester supplementation reduces peripheral symptoms in a mouse model of Niemann-Pick type C1 disease. | 2020 Jun |
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| 18 |
32371566 | Mass spectrometry imaging and LC/MS reveal decreased cerebellar phosphoinositides in Niemann-Pick type C1-null mice. | 2020 Jul |
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| 19 |
32599915 | Identification of Brain-Specific Treatment Effects in NPC1 Disease by Focusing on Cellular and Molecular Changes of Sphingosine-1-Phosphate Metabolism. | 2020 Jun 24 |
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| 20 |
32731618 | Single Cell Transcriptome Analysis of Niemann-Pick Disease, Type C1 Cerebella. | 2020 Jul 28 |
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| 21 |
32770132 | Plasmid DNA gene therapy of the Niemann-Pick C1 mouse with transferrin receptor-targeted Trojan horse liposomes. | 2020 Aug 7 |
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| 22 |
29900534 | Absence of infiltrating peripheral myeloid cells in the brains of mouse models of lysosomal storage disorders. | 2019 Mar |
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| 23 |
30923761 | Hepatic and neuronal phenotype of NPC1-/- mice. | 2019 Mar |
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| 24 |
31178699 | Anomalies in Dopamine Transporter Expression and Primary Cilium Distribution in the Dorsal Striatum of a Mouse Model of Niemann-Pick C1 Disease. | 2019 |
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31535451 | NPC1 enables cholesterol mobilization during long-term potentiation that can be restored in Niemann-Pick disease type C by CYP46A1 activation. | 2019 Nov 5 |
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| 26 |
31628414 | Hematopoietic Npc1 mutation shifts gut microbiota composition in Ldlr-/- mice on a high-fat, high-cholesterol diet. | 2019 Oct 18 |
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| 27 |
31641049 | Sphingosine Kinase 2 Potentiates Amyloid Deposition but Protects against Hippocampal Volume Loss and Demyelination in a Mouse Model of Alzheimer's Disease. | 2019 Nov 27 |
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| 28 |
31711490 | Synthetic high-density lipoprotein nanoparticles for the treatment of Niemann-Pick diseases. | 2019 Nov 11 |
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| 29 |
29052144 | The Effects of Extracellular Serum Concentration on APP Processing in Npc1-Deficient APP-Overexpressing N2a Cells. | 2018 Jul |
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| 30 |
29587349 | Evaluation of Two Liver Treatment Strategies in a Mouse Model of Niemann-Pick-Disease Type C1. | 2018 Mar 24 |
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29878847 | Niemann-Pick C1-deficient mice lacking sterol O-acyltransferase 2 have less hepatic cholesterol entrapment and improved liver function. | 2018 Oct 1 |
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30249300 | The susceptibility of cochlear outer hair cells to cyclodextrin is not related to their electromotile activity. | 2018 Sep 24 |
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| 33 |
30666257 | Pneumococcal Immunization Reduces Neurological and Hepatic Symptoms in a Mouse Model for Niemann-Pick Type C1 Disease. | 2018 |
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| 34 |
27798114 | Systemic AAV9 gene therapy improves the lifespan of mice with Niemann-Pick disease, type C1. | 2017 Jan 1 |
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| 35 |
28262793 | Characterization of cholesterol homeostasis in sphingosine-1-phosphate lyase-deficient fibroblasts reveals a Niemann-Pick disease type C-like phenotype with enhanced lysosomal Ca2+ storage. | 2017 Mar 6 |
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| 36 |
28379564 | Shortened primary cilium length and dysregulated Sonic hedgehog signaling in Niemann-Pick C1 disease. | 2017 Jun 15 |
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| 37 |
28487438 | The Niemann-Pick C1 gene interacts with a high-fat diet to promote weight gain through differential regulation of central energy metabolism pathways. | 2017 Aug 1 |
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| 38 |
26467605 | Circadian profiling in two mouse models of lysosomal storage disorders; Niemann Pick type-C and Sandhoff disease. | 2016 Jan 15 |
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| 39 |
26771826 | Gpnmb Is a Potential Marker for the Visceral Pathology in Niemann-Pick Type C Disease. | 2016 |
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| 40 |
26903308 | Susceptibility of outer hair cells to cholesterol chelator 2-hydroxypropyl-β-cyclodextrine is prestin-dependent. | 2016 Feb 23 |
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| 41 |
27083515 | Presymptomatic Alterations in Amino Acid Metabolism and DNA Methylation in the Cerebellum of a Murine Model of Niemann-Pick Type C Disease. | 2016 Jun |
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| 42 |
27816810 | Prevention of oxLDL uptake leads to decreased atherosclerosis in hematopoietic NPC1-deficient Ldlr-/- mice. | 2016 Dec |
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| 43 |
27834854 | Pharmacologic Treatment Assigned for Niemann Pick Type C1 Disease Partly Changes Behavioral Traits in Wild-Type Mice. | 2016 Nov 9 |
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26015498 | Niemann-pick C1 is essential for ebolavirus replication and pathogenesis in vivo. | 2015 May 26 |
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| 45 |
26019327 | A murine Niemann-Pick C1 I1061T knock-in model recapitulates the pathological features of the most prevalent human disease allele. | 2015 May 27 |
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| 46 |
26027824 | Efficacy of 2-Hydroxypropyl-β-cyclodextrin in Niemann-Pick Disease Type C Model Mice and Its Pharmacokinetic Analysis in a Patient with the Disease. | 2015 |
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| 47 |
24076310 | Ontogenic changes in lung cholesterol metabolism, lipid content, and histology in mice with Niemann-Pick type C disease. | 2014 Jan |
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| 48 |
24910948 | Relevance of neuronal and glial NPC1 for synaptic input to cerebellar Purkinje cells. | 2014 Jul |
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| 49 |
25009206 | Dysregulation of testicular cholesterol metabolism following spontaneous mutation of the niemann-pick c1 gene in mice. | 2014 Aug |
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25115571 | Systemic administration of 2-hydroxypropyl-β-cyclodextrin to symptomatic Npc1-deficient mice slows cholesterol sequestration in the major organs and improves liver function. | 2014 Oct |
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