These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.
3. Systemic administration of 2-hydroxypropyl-β-cyclodextrin to symptomatic Npc1-deficient mice slows cholesterol sequestration in the major organs and improves liver function. Lopez AM; Terpack SJ; Posey KS; Liu B; Ramirez CM; Turley SD Clin Exp Pharmacol Physiol; 2014 Oct; 41(10):780-7. PubMed ID: 25115571 [TBL] [Abstract][Full Text] [Related]
4. Identification of Niemann-Pick C1 disease biomarkers through sphingolipid profiling. Fan M; Sidhu R; Fujiwara H; Tortelli B; Zhang J; Davidson C; Walkley SU; Bagel JH; Vite C; Yanjanin NM; Porter FD; Schaffer JE; Ory DS J Lipid Res; 2013 Oct; 54(10):2800-14. PubMed ID: 23881911 [TBL] [Abstract][Full Text] [Related]
5. Amyloid-β metabolism in Niemann-Pick C disease models and patients. Mattsson N; Olsson M; Gustavsson MK; Kosicek M; Malnar M; Månsson JE; Blomqvist M; Gobom J; Andreasson U; Brinkmalm G; Vite C; Hecimovic S; Hastings C; Blennow K; Zetterberg H; Portelius E Metab Brain Dis; 2012 Dec; 27(4):573-85. PubMed ID: 22935999 [TBL] [Abstract][Full Text] [Related]
6. Weekly cyclodextrin administration normalizes cholesterol metabolism in nearly every organ of the Niemann-Pick type C1 mouse and markedly prolongs life. Ramirez CM; Liu B; Taylor AM; Repa JJ; Burns DK; Weinberg AG; Turley SD; Dietschy JM Pediatr Res; 2010 Oct; 68(4):309-15. PubMed ID: 20581737 [TBL] [Abstract][Full Text] [Related]
7. Visual evoked potentials of Niemann-Pick type C1 mice reveal an impairment of the visual pathway that is rescued by 2-hydroxypropyl-ß-cyclodextrin. Palladino G; Loizzo S; Fortuna A; Canterini S; Palombi F; Erickson RP; Mangia F; Fiorenza MT Orphanet J Rare Dis; 2015 Oct; 10():133. PubMed ID: 26458950 [TBL] [Abstract][Full Text] [Related]
8. 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. Ashikawa H; Mogi H; Honda T; Nakamura H; Murayama T Eur J Pharmacol; 2021 Apr; 896():173907. PubMed ID: 33503462 [TBL] [Abstract][Full Text] [Related]
9. Improved neuroprotection using miglustat, curcumin and ibuprofen as a triple combination therapy in Niemann-Pick disease type C1 mice. Williams IM; Wallom KL; Smith DA; Al Eisa N; Smith C; Platt FM Neurobiol Dis; 2014 Jul; 67():9-17. PubMed ID: 24631719 [TBL] [Abstract][Full Text] [Related]
10. Altered levels and distribution of amyloid precursor protein and its processing enzymes in Niemann-Pick type C1-deficient mouse brains. Kodam A; Maulik M; Peake K; Amritraj A; Vetrivel KS; Thinakaran G; Vance JE; Kar S Glia; 2010 Aug; 58(11):1267-81. PubMed ID: 20607864 [TBL] [Abstract][Full Text] [Related]
12. Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression. Davidson CD; Ali NF; Micsenyi MC; Stephney G; Renault S; Dobrenis K; Ory DS; Vanier MT; Walkley SU PLoS One; 2009 Sep; 4(9):e6951. PubMed ID: 19750228 [TBL] [Abstract][Full Text] [Related]
13. Normalization of cholesterol homeostasis by 2-hydroxypropyl-β-cyclodextrin in neurons and glia from Niemann-Pick C1 (NPC1)-deficient mice. Peake KB; Vance JE J Biol Chem; 2012 Mar; 287(12):9290-8. PubMed ID: 22277650 [TBL] [Abstract][Full Text] [Related]
14. Relative acidic compartment volume as a lysosomal storage disorder-associated biomarker. te Vruchte D; Speak AO; Wallom KL; Al Eisa N; Smith DA; Hendriksz CJ; Simmons L; Lachmann RH; Cousins A; Hartung R; Mengel E; Runz H; Beck M; Amraoui Y; Imrie J; Jacklin E; Riddick K; Yanjanin NM; Wassif CA; Rolfs A; Rimmele F; Wright N; Taylor C; Ramaswami U; Cox TM; Hastings C; Jiang X; Sidhu R; Ory DS; Arias B; Jeyakumar M; Sillence DJ; Wraith JE; Porter FD; Cortina-Borja M; Platt FM J Clin Invest; 2014 Mar; 124(3):1320-8. PubMed ID: 24487591 [TBL] [Abstract][Full Text] [Related]
15. Hydroxypropyl-beta and -gamma cyclodextrins rescue cholesterol accumulation in Niemann-Pick C1 mutant cell via lysosome-associated membrane protein 1. Singhal A; Szente L; Hildreth JEK; Song B Cell Death Dis; 2018 Oct; 9(10):1019. PubMed ID: 30282967 [TBL] [Abstract][Full Text] [Related]
16. Pulmonary abnormalities in animal models due to Niemann-Pick type C1 (NPC1) or C2 (NPC2) disease. Roszell BR; Tao JQ; Yu KJ; Gao L; Huang S; Ning Y; Feinstein SI; Vite CH; Bates SR PLoS One; 2013; 8(7):e67084. PubMed ID: 23843985 [TBL] [Abstract][Full Text] [Related]
17. Accumulation of alkyl-lysophosphatidylcholines in Niemann-Pick disease type C1. Mishra S; Kell P; Scherrer D; Dietzen DJ; Vite CH; Berry-Kravis E; Davidson C; Cologna SM; Porter FD; Ory DS; Jiang X J Lipid Res; 2024 Aug; 65(8):100600. PubMed ID: 39048052 [TBL] [Abstract][Full Text] [Related]
18. Evaluation of Two Liver Treatment Strategies in a Mouse Model of Niemann-Pick-Disease Type C1. Ebner L; Gläser A; Bräuer A; Witt M; Wree A; Rolfs A; Frank M; Vollmar B; Kuhla A Int J Mol Sci; 2018 Mar; 19(4):. PubMed ID: 29587349 [TBL] [Abstract][Full Text] [Related]
19. Ontogenic changes in lung cholesterol metabolism, lipid content, and histology in mice with Niemann-Pick type C disease. Ramirez CM; Lopez AM; Le LQ; Posey KS; Weinberg AG; Turley SD Biochim Biophys Acta; 2014 Jan; 1841(1):54-61. PubMed ID: 24076310 [TBL] [Abstract][Full Text] [Related]
20. Intracerebroventricular Treatment with 2-Hydroxypropyl-β-Cyclodextrin Decreased Cerebellar and Hepatic Glycoprotein Nonmetastatic Melanoma Protein B (GPNMB) Expression in Niemann-Pick Disease Type C Model Mice. Fukaura M; Ishitsuka Y; Shirakawa S; Ushihama N; Yamada Y; Kondo Y; Takeo T; Nakagata N; Motoyama K; Higashi T; Arima H; Kurauchi Y; Seki T; Katsuki H; Higaki K; Matsuo M; Irie T Int J Mol Sci; 2021 Jan; 22(1):. PubMed ID: 33466390 [TBL] [Abstract][Full Text] [Related] [Next] [New Search]