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.


BIOMARKERS

Molecular Biopsy of Human Tumors

- a resource for Precision Medicine *

300 related articles for article (PubMed ID: 29223359)

  • 1. In Niemann-Pick C1 mouse models, glial-only expression of the normal gene extends survival much further than do changes in genetic background or treatment with hydroxypropyl-beta-cyclodextrin.
    Marshall CA; Watkins-Chow DE; Palladino G; Deutsch G; Chandran K; Pavan WJ; Erickson RP
    Gene; 2018 Feb; 643():117-123. PubMed ID: 29223359
    [TBL] [Abstract][Full Text] [Related]  

  • 2. Developmental delay in motor skill acquisition in Niemann-Pick C1 mice reveals abnormal cerebellar morphogenesis.
    Caporali P; Bruno F; Palladino G; Dragotto J; Petrosini L; Mangia F; Erickson RP; Canterini S; Fiorenza MT
    Acta Neuropathol Commun; 2016 Sep; 4(1):94. PubMed ID: 27586038
    [TBL] [Abstract][Full Text] [Related]  

  • 3. Decreased neural stem cell proliferation and olfaction in mouse models of Niemann-Pick C1 disease and the response to hydroxypropyl-β-cyclodextrin.
    Dragotto J; Palladino G; Canterini S; Caporali P; Patil R; Fiorenza MT; Erickson RP
    J Appl Genet; 2019 Nov; 60(3-4):357-365. PubMed ID: 31485950
    [TBL] [Abstract][Full Text] [Related]  

  • 4. A novel mouse model of Niemann-Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations.
    Maue RA; Burgess RW; Wang B; Wooley CM; Seburn KL; Vanier MT; Rogers MA; Chang CC; Chang TY; Harris BT; Graber DJ; Penatti CA; Porter DM; Szwergold BS; Henderson LP; Totenhagen JW; Trouard TP; Borbon IA; Erickson RP
    Hum Mol Genet; 2012 Feb; 21(4):730-50. PubMed ID: 22048958
    [TBL] [Abstract][Full Text] [Related]  

  • 5. Necroptosis inhibition as a therapy for Niemann-Pick disease, type C1: Inhibition of RIP kinases and combination therapy with 2-hydroxypropyl-β-cyclodextrin.
    Cougnoux A; Clifford S; Salman A; Ng SL; Bertin J; Porter FD
    Mol Genet Metab; 2018 Dec; 125(4):345-350. PubMed ID: 30392741
    [TBL] [Abstract][Full Text] [Related]  

  • 6. The susceptibility of cochlear outer hair cells to cyclodextrin is not related to their electromotile activity.
    Zhou Y; Takahashi S; Homma K; Duan C; Zheng J; Cheatham MA; Zheng J
    Acta Neuropathol Commun; 2018 Sep; 6(1):98. PubMed ID: 30249300
    [TBL] [Abstract][Full Text] [Related]  

  • 7. A pilot study of direct delivery of hydroxypropyl-beta-cyclodextrin to the lung by the nasal route in a mouse model of Niemann-Pick C1 disease: motor performance is unaltered and lung disease is worsened.
    Erickson RP; Deutsch G; Patil R
    J Appl Genet; 2018 May; 59(2):187-191. PubMed ID: 29411332
    [TBL] [Abstract][Full Text] [Related]  

  • 8. Genetic background modifies phenotypic severity and longevity in a mouse model of Niemann-Pick disease type C1.
    Rodriguez-Gil JL; Watkins-Chow DE; Baxter LL; Elliot G; Harper UL; Wincovitch SM; Wedel JC; Incao AA; Huebecker M; Boehm FJ; Garver WS; Porter FD; Broman KW; Platt FM; Pavan WJ
    Dis Model Mech; 2020 Mar; 13(3):. PubMed ID: 31996359
    [TBL] [Abstract][Full Text] [Related]  

  • 9. 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]  

  • 10. 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]  

  • 11. 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]  

  • 12. 2-Hydroxypropyl-β-cyclodextrin is the active component in a triple combination formulation for treatment of Niemann-Pick C1 disease.
    Davidson J; Molitor E; Moores S; Gale SE; Subramanian K; Jiang X; Sidhu R; Kell P; Zhang J; Fujiwara H; Davidson C; Helquist P; Melancon BJ; Grigalunas M; Liu G; Salahi F; Wiest O; Xu X; Porter FD; Pipalia NH; Cruz DL; Holson EB; Schaffer JE; Walkley SU; Maxfield FR; Ory DS
    Biochim Biophys Acta Mol Cell Biol Lipids; 2019 Oct; 1864(10):1545-1561. PubMed ID: 31051283
    [TBL] [Abstract][Full Text] [Related]  

  • 13. 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]  

  • 14. The role of decreased levels of Niemann-Pick C1 intracellular cholesterol transport on obesity is reversed in the C57BL/6J, metabolic syndrome mouse strain: a metabolic or an inflammatory effect?
    Borbon I; Campbell E; Ke W; Erickson RP
    J Appl Genet; 2012 Aug; 53(3):323-30. PubMed ID: 22585185
    [TBL] [Abstract][Full Text] [Related]  

  • 15. Npc1 deficiency in the C57BL/6J genetic background enhances Niemann-Pick disease type C spleen pathology.
    Parra J; Klein AD; Castro J; Morales MG; Mosqueira M; Valencia I; Cortés V; Rigotti A; Zanlungo S
    Biochem Biophys Res Commun; 2011 Sep; 413(3):400-6. PubMed ID: 21910975
    [TBL] [Abstract][Full Text] [Related]  

  • 16. Amelioration of enteric neuropathology in a mouse model of Niemann-Pick C by Npc1 expression in enteric glia.
    Kapur R; Donohue C; Jelinek D; Erickson RP
    J Neurosci Res; 2009 Oct; 87(13):2994-3001. PubMed ID: 19472223
    [TBL] [Abstract][Full Text] [Related]  

  • 17. Ontogenesis and Modulation of Intestinal Unesterified Cholesterol Sequestration in a Mouse Model of Niemann-Pick C1 Disease.
    Lopez AM; Ramirez CM; Taylor AM; Jones RD; Repa JJ; Turley SD
    Dig Dis Sci; 2020 Jan; 65(1):158-167. PubMed ID: 31312996
    [TBL] [Abstract][Full Text] [Related]  

  • 18. 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]  

  • 19. The C57BL/6J Niemann-Pick C1 mouse model with decreased gene dosage has impaired glucose tolerance independent of body weight.
    Jelinek D; Castillo JJ; Garver WS
    Gene; 2013 Sep; 527(1):65-70. PubMed ID: 23769925
    [TBL] [Abstract][Full Text] [Related]  

  • 20. Systemic AAV9 gene therapy improves the lifespan of mice with Niemann-Pick disease, type C1.
    Chandler RJ; Williams IM; Gibson AL; Davidson CD; Incao AA; Hubbard BT; Porter FD; Pavan WJ; Venditti CP
    Hum Mol Genet; 2017 Jan; 26(1):52-64. PubMed ID: 27798114
    [TBL] [Abstract][Full Text] [Related]  

    [Next]    [New Search]
    of 15.