Cyclodextrins in the treatment of a mouse model of Niemann-Pick C disease

Life Sciences

Volumn 70, Issue 2, 2001, Pages 131-142

  Camargo, Fernando ^a   Erickson, Robert P ^a   Garver, William S ^c   Hossain, G Showkat ^c   Carbone, Peter N ^a   Heidenreich, Randall A ^a   Blanchard, James ^b  

^a UNIVERSITY OF ARIZONA COLLEGE OF MEDICINE   (United States)

^b UNIVERSITY OF ARIZONA   (United States)

^c Department of Pediatrics ^*

Author keywords

Cholesterol; Mouse models; Neurodegenerative disorders

Indexed keywords

2 HYDROXYPROPYL BETA CYCLODEXTRIN; CHOLESTEROL; NIFEDIPINE; PROBUCOL; TRACER;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BLOOD BRAIN BARRIER; CHOLESTEROL ESTERIFICATION; CHOLESTEROL LIVER LEVEL; CONTROLLED STUDY; DRUG ABSORPTION; DRUG EFFECT; DRUG EFFICACY; DRUG EXPOSURE; DRUG LABELING; GENE DISRUPTION; KNOCKOUT GENE; KNOCKOUT MOUSE; MOUSE; NEUROLOGIC DISEASE; NIEMANN PICK DISEASE; NONHUMAN; OSMOTIC MINIPUMP; PLACENTAL TRANSFER; STRUCTURE ACTIVITY RELATION;

ANIMALS; ANTICHOLESTEREMIC AGENTS; ATAXIA; BETA-CYCLODEXTRINS; BLOOD-BRAIN BARRIER; BRAIN; CHOLESTEROL; CYCLODEXTRINS; DISEASE MODELS, ANIMAL; DOSE-RESPONSE RELATIONSHIP, DRUG; DRUG THERAPY, COMBINATION; FEMALE; INJECTIONS, INTRAPERITONEAL; INJECTIONS, SPINAL; LIVER; MALE; MICE; MICE, KNOCKOUT; NIEMANN-PICK DISEASES; NIFEDIPINE; PROBUCOL; PROTEINS; TREMOR;

ANIMALIA;

EID: 0035976885     PISSN: 00243205     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0024-3205(01)01384-4     Document Type: Article

References (31)

1. Niemann-Pick C disease: Recent advances
2. Niemann-Pick C1 disease gene: Homology to mediators of cholesterol homeostasis
3. Murine model of Niemann-Pick C disease: Mutation in a cholesterol homeosta-sis gene
4. Cholesterol and oxygenated cholesterol concentrations are markedly elevated in peripheral tissue but not in brain from mice with the Niemann-Pick type C phenotype
5. Correction of sphingomyelinase deficiency in Niemann-Pick type C fibroblasts by removal of lipoprotein fraction from culture media
6. The effect of cholesterol-lowering agents on hepatic and plasma cholesterol in Niemann-Pick disease type C
7. Clinical improvement with DMSO treatment in a patient with Niemann-Pick disease (type C)
8. Magnetic resonance spectroscopy in Niemann-Pick disease type C: Correlation with diagnosis and clinical response to cholestyramine and lovastatin
9. Pharmacological and genetic modifi-cations of somatic cholesterol do not substantially alter the course of CNS disease in Niemann-Pick C mice
10. Differential effects of alpha, beta and gamma-cyclodex-trins on human erythrocytes
11. Hydroxypropyl-cyclodextrins in parenteral use. I: Lipid dissolution and effects on lipid transfers in vitro
12. Characterization of an inclusion complex of cholesterol and hydroxypropyl-beta-cyclodextrin
13. Cellular cholesterol efflux mediated by cyclodextrins
14. Cyclodextrins as catalysts for the removal of cholesterol from macrophage foam cells
15. The effect of parenterally administered cyclodextrins on cholesterol levels in the rat
16. Hypocholesterolemic action of β-cyclodextrin and its effects on cholesterol metabolism in pigs fed a cholesterol-enriched diet
17. Disruption of the mouse mdr1a P-glycoprotein gene leads to a deficiency in the blood-brain barrier and to increased sensitivity to drugs
18. Some important considerations in the use of cyclodextrins
19. Lipoprotein oxidation and cytotoxicity: Effect of probucol on streptozotocin-treated rats
20. The picomole determination of free and total cholesterol in cells in culture
21. Intra-cellular trafficking of cholesterol monitored with a cyclodextrin
22. Cyclodextrins - Enabling excipients: Their present and future use in pharmaceuticals
23. Altered expression of caveolin-1 and increased cholesterol in detergent insoluble membrane fractions from liver in mice with Niemann-Pick disease type C
24. The caveolae membrane system
25. The biochemistry of P-glycoprotein-mediated multidrug resistance
26. P-glycoprotein in the blood-brain barrier of mice influences the brain penetration and pharmacological activity of many drugs
27. Parenteral hydroxypropyl cyclodextrins: Intravenous and intracerebral administration of lipophils
28. Pathway across blood-brain barrier opened by the bradykinin agonist, RMP-7
29. Controlled modulation of BBB permeability using the bradykinin agonist, RMP-7
30. Intravenous infusion of RMP-7 increases ocular uptake of ganciclovir