Unraveling the sterol-trafficking defect in Niemann-Pick C disease
- PMID: 19211789
- PMCID: PMC2650112
- DOI: 10.1073/pnas.0812934106
Unraveling the sterol-trafficking defect in Niemann-Pick C disease
Conflict of interest statement
The authors declare no conflict of interest.
Comment on
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Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse.Proc Natl Acad Sci U S A. 2009 Feb 17;106(7):2377-82. doi: 10.1073/pnas.0810895106. Epub 2009 Jan 26. Proc Natl Acad Sci U S A. 2009. PMID: 19171898 Free PMC article.
References
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- Infante RE, et al. Purified NPC1 protein. I. Binding of cholesterol and oxysterols to a 1278-amino acid membrane protein. J Biol Chem. 2008;283:1052–1063. - PubMed
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- Liou HL, et al. NPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterols. J Biol Chem. 2006;281:36710–36723. - PubMed
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