Journal article

Lipids on trial: the search for the offending metabolite in Niemann-Pick type C disease.

Abstract:: Niemann-Pick disease type C is a complex lysosomal storage disorder caused by mutations in either the NPC1 or NPC2 genes that is characterized at the cellular level by the storage of multiple lipids, defective lysosomal calcium homeostasis and unique trafficking defects. We review the potential role of each of the individual storage lipids in initiating the pathogenic cascade and propose a model of NPC1 and NPC2 function based on the current knowledge.

Publication status:: Published

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APA Style

Lloyd-Evans, E., & Platt, F. (2010). Lipids on trial: the search for the offending metabolite in Niemann-Pick type C disease. Traffic (Copenhagen, Denmark), 11(4), 419–428.

MLA Style

Lloyd-Evans, E, and F Platt. “Lipids on Trial: the Search for the Offending Metabolite in Niemann-Pick Type C Disease.” Traffic (Copenhagen, Denmark), vol. 11, no. 4, 2010, pp. 419–28.

Chicago Style

Lloyd-Evans, E, and F Platt. 2010. “Lipids on Trial: the Search for the Offending Metabolite in Niemann-Pick Type C Disease.” Traffic (Copenhagen, Denmark) 11 (4): 419–28.
Print

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Publisher copy:: 10.1111/j.1600-0854.2010.01032.x

Authors

+ Lloyd-Evans, E More by this author

Role:: Author

+ Platt, F More by this author

Institution:: University of Oxford
Division:: MSD
Department:: Pharmacology
Role:: Author

Journal:: Traffic (Copenhagen, Denmark) More from this journal
Volume:: 11
Issue:: 4
Pages:: 419-428
Publication date:: 2010-04-01
DOI:: 10.1111/j.1600-0854.2010.01032.x
EISSN:: 1600-0854
ISSN:: 1398-9219

Language:: English
Keywords:: Glycosphingolipids

Membrane Glycoproteins

Mice

Carrier Proteins

Lipid Metabolism

Animals

Sphingosine

Cholesterol

Humans

Niemann-Pick Disease, Type C

Glycoproteins

Lysosomes
Pubs id:: pubs:185198
UUID:: uuid:1d74003a-97e0-4981-a489-160ede5d7e28
Local pid:: pubs:185198
Source identifiers:: 185198
Deposit date:: 2012-12-19
ARK identifier:: ark:/29072/ora_1d74003a97e04981a489160ede5d7e28

Terms of use

Copyright date:: 2010

Licence:: Terms and Conditions of Use for Oxford University Research Archive

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