Literature DB >> 19211789

Unraveling the sterol-trafficking defect in Niemann-Pick C disease.

Stephen L Sturley1, Marc C Patterson, Peter Pentchev.   

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Year:  2009        PMID： 19211789      PMCID： PMC2650112          DOI： 10.1073/pnas.0812934106

Source DB:  PubMed          Journal:  Proc Natl Acad Sci U S A        ISSN： 0027-8424            Impact factor:   11.205


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  10 in total

1.  Lysosomal unesterified cholesterol content correlates with liver cell death in murine Niemann-Pick type C disease.

Authors:  Eduardo P Beltroy; Benny Liu; John M Dietschy; Stephen D Turley
Journal:  J Lipid Res       Date:  2007-01-14       Impact factor: 5.922

2.  Purified NPC1 protein. I. Binding of cholesterol and oxysterols to a 1278-amino acid membrane protein.

Authors:  Rodney E Infante; Lina Abi-Mosleh; Arun Radhakrishnan; Jarrod D Dale; Michael S Brown; Joseph L Goldstein
Journal:  J Biol Chem       Date:  2007-11-06       Impact factor: 5.157

3.  NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes.

Authors:  Rodney E Infante; Michael L Wang; Arun Radhakrishnan; Hyock Joo Kwon; Michael S Brown; Joseph L Goldstein
Journal:  Proc Natl Acad Sci U S A       Date:  2008-09-04       Impact factor: 11.205

4.  Intracellular trafficking of the free cholesterol derived from LDL cholesteryl ester is defective in vivo in Niemann-Pick C disease: insights on normal metabolism of HDL and LDL gained from the NP-C mutation.

Authors:  R D Shamburek; P G Pentchev; L A Zech; J Blanchette-Mackie; E D Carstea; J M VandenBroek; P S Cooper; E B Neufeld; R D Phair; H B Brewer; R O Brady; C C Schwartz
Journal:  J Lipid Res       Date:  1997-12       Impact factor: 5.922

5.  Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse.

Authors:  Benny Liu; Stephen D Turley; Dennis K Burns; Anna M Miller; Joyce J Repa; John M Dietschy
Journal:  Proc Natl Acad Sci U S A       Date:  2009-01-26       Impact factor: 11.205

6.  Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport.

Authors:  David E Sleat; Jennifer A Wiseman; Mukarram El-Banna; Sandy M Price; Lucie Verot; Michael M Shen; G Stephen Tint; Marie T Vanier; Steven U Walkley; Peter Lobel
Journal:  Proc Natl Acad Sci U S A       Date:  2004-04-07       Impact factor: 11.205

7.  Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse.

Authors:  Joyce J Repa; Hao Li; Tamy C Frank-Cannon; Mark A Valasek; Stephen D Turley; Malú G Tansey; John M Dietschy
Journal:  J Neurosci       Date:  2007-12-26       Impact factor: 6.167

8.  Genetic variations and treatments that affect the lifespan of the NPC1 mouse.

Authors:  Benny Liu; Hao Li; Joyce J Repa; Stephen D Turley; John M Dietschy
Journal:  J Lipid Res       Date:  2007-12-12       Impact factor: 5.922

9.  NPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterols.

Authors:  Heng-Ling Liou; Sayali S Dixit; Sujuan Xu; G Stephen Tint; Ann M Stock; Peter Lobel
Journal:  J Biol Chem       Date:  2006-10-02       Impact factor: 5.157

10.  Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium.

Authors:  Emyr Lloyd-Evans; Anthony J Morgan; Xingxuan He; David A Smith; Elena Elliot-Smith; Daniel J Sillence; Grant C Churchill; Edward H Schuchman; Antony Galione; Frances M Platt
Journal:  Nat Med       Date:  2008-10-26       Impact factor: 53.440
  10 in total
  13 in total

1.  A sensitive and specific LC-MS/MS method for rapid diagnosis of Niemann-Pick C1 disease from human plasma.

Authors:  Xuntian Jiang; Rohini Sidhu; Forbes D Porter; Nicole M Yanjanin; Anneliese O Speak; Danielle Taylor te Vruchte; Frances M Platt; Hideji Fujiwara; David E Scherrer; Jessie Zhang; Dennis J Dietzen; Jean E Schaffer; Daniel S Ory
Journal:  J Lipid Res       Date:  2011-04-24       Impact factor: 5.922

2.  Cholesterol oxidation products are sensitive and specific blood-based biomarkers for Niemann-Pick C1 disease.

Authors:  Forbes D Porter; David E Scherrer; Michael H Lanier; S Joshua Langmade; Vasumathi Molugu; Sarah E Gale; Dana Olzeski; Rohini Sidhu; Dennis J Dietzen; Rao Fu; Christopher A Wassif; Nicole M Yanjanin; Steven P Marso; John House; Charles Vite; Jean E Schaffer; Daniel S Ory
Journal:  Sci Transl Med       Date:  2010-11-03       Impact factor: 17.956

Review 3.  24(S)-Hydroxycholesterol as a Modulator of Neuronal Signaling and Survival.

Authors:  Min-Yu Sun; Andrew J Linsenbardt; Christine M Emnett; Lawrence N Eisenman; Yukitoshi Izumi; Charles F Zorumski; Steve Mennerick
Journal:  Neuroscientist       Date:  2015-01-27       Impact factor: 7.519

Review 4.  Molecular pathways for intracellular cholesterol accumulation: common pathogenic mechanisms in Niemann-Pick disease Type C and cystic fibrosis.

Authors:  Nicholas L Cianciola; Cathleen R Carlin; Thomas J Kelley
Journal:  Arch Biochem Biophys       Date:  2011-09-05       Impact factor: 4.013

Review 5.  The Extending Spectrum of NPC1-Related Human Disorders: From Niemann-Pick C1 Disease to Obesity.

Authors:  Amel Lamri; Marie Pigeyre; William S Garver; David Meyre
Journal:  Endocr Rev       Date:  2018-04-01       Impact factor: 19.871

6.  Thiadiazole carbamates: potent inhibitors of lysosomal acid lipase and potential Niemann-Pick type C disease therapeutics.

Authors:  Anton I Rosenbaum; Casey C Cosner; Christopher J Mariani; Frederick R Maxfield; Olaf Wiest; Paul Helquist
Journal:  J Med Chem       Date:  2010-07-22       Impact factor: 7.446

Review 7.  Sterols in spermatogenesis and sperm maturation.

Authors:  Rok Keber; Damjana Rozman; Simon Horvat
Journal:  J Lipid Res       Date:  2012-10-23       Impact factor: 5.922

Review 8.  Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin.

Authors:  Jean E Vance; Barbara Karten
Journal:  J Lipid Res       Date:  2014-03-24       Impact factor: 5.922

9.  Mathematical modeling and validation of the ergosterol pathway in Saccharomyces cerevisiae.

Authors:  Fernando Alvarez-Vasquez; Howard Riezman; Yusuf A Hannun; Eberhard O Voit
Journal:  PLoS One       Date:  2011-12-14       Impact factor: 3.240

10.  Cholesterol-dependent energy transfer between fluorescent proteins-insights into protein proximity of APP and BACE1 in different membranes in Niemann-Pick type C disease cells.

Authors:  Bjoern von Einem; Petra Weber; Michael Wagner; Martina Malnar; Marko Kosicek; Silva Hecimovic; Christine A F von Arnim; Herbert Schneckenburger
Journal:  Int J Mol Sci       Date:  2012-11-26       Impact factor: 5.923
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