Systemic administration of 2-hydroxypropyl-β-cyclodextrin to symptomatic Npc1-deficient mice slows cholesterol sequestration in the major organs and improves liver function-Reference-Cited by-同舟云学术

Systemic administration of 2-hydroxypropyl-β-cyclodextrin to symptomatic Npc1-deficient mice slows cholesterol sequestration in the major organs and improves liver function

Published:2014-10 Issue:10 Volume:41 Page:780-787
ISSN:0305-1870
Container-title:Clinical and Experimental Pharmacology and Physiology
language:en
Short-container-title:Clin Exp Pharmacol Physiol

Author:

Lopez Adam M¹,Terpack Sandi J²,Posey Kenneth S¹,Liu Benny¹,Ramirez Charina M³,Turley Stephen D¹

Affiliation:

1. Department of Internal Medicine; University of Texas Southwestern Medical Center; Dallas TX USA

2. Department of Biochemistry; University of Texas Southwestern Medical Center; Dallas TX USA

3. Department of Pediatrics; University of Texas Southwestern Medical Center; Dallas TX USA

Funder

National Institutes of Health

Publisher

Wiley

Subject

Physiology (medical),Pharmacology,Physiology

Link

http://onlinelibrary.wiley.com/wol1/doi/10.1111/1440-1681.12285/fullpdf

Reference34 articles.

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2. Amino acid substitution in NPC1 that abolishes cholesterol binding reproduces phenotype of complete NPC1 deficiency in mice;Xie;Proc. Natl. Acad. Sci. U.S.A.,2011

3. Current controversies in Niemann-Pick C1 disease: Steroids or gangliosides; neurons or neurons and glia;Erickson;J. Appl. Genet.,2013

4. Current status of drug therapy development for Niemann-Pick type C disease;Helquist;Drugs Fut.,2009

5. Therapeutic potential of cyclodextrins in the treatment of Niemann-Pick type C disease;Liu;Clin. Lipidol.,2012

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