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Molecular BioSystems Vol - 9 Issue - 3

Molecular BioSystems Vol - 9 Issue - 2

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Molecular BioSystems Vol -4

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Molecular BioSystems Vol -2

Interaction of AnxA6 with isolated and artificial lipid microdomains; importance of lipid composition and calcium content

Content Provider	Royal Society of Chemistry (RSC)
Author	Besson, Françoise Bandorowicz-Pikula, Joanna Domon, Magdalena M. Tylki-Szymanska, Anna Pikula, Slawomir
Copyright Year	2013
Abstract	Niemann-Pick type C (NPC) disease is a lipid storage disorder characterized by accumulation of lipids in the late endosome/lysosome (LE/LY) compartment. In our previous report we isolated membranes of the LE/LY compartment from NPC L1 skin fibroblasts with a mutation in the NPC1 gene and found that they were characterized by low fluidity which likely contributed to the impaired function of membrane proteins involved in storage and turnover of cholesterol. In this report we isolated lipid microdomains (DRMs) from membranes of various cellular compartments and observed an increased amount of DRMs in the LE/LY compartment of NPC L1 cells in comparison to control cells, with no change in the DRM content in the plasma membrane. In addition, in the NPC cells, the majority of the cholesterol-interacting protein, AnxA6, which participates in the transport and distribution of cholesterol, translocated to DRMs upon a rise in Ca2+ concentration. The mechanism of this translocation was further studied in vitro using Langmuir monolayers. We found that Ca2+ is the main factor which regulates the interaction of AnxA6 with monolayers composed of neutral lipids, such as DPPC and sphingomyelin, and may also determine AnxA6 localization in cholesterol and sphingomyelin enriched microdomains, thus contributing to the etiology of the NPC disease.
Starting Page	668
Ending Page	676
Page Count	9
File Format	HTM / HTML PDF
ISSN	1742206X
Volume Number	9
Issue Number	4
Journal	Molecular BioSystems
DOI	10.1039/c3mb25487a
Language	English
Publisher	Royal Society of Chemistry
Access Restriction	Open
Subject Keyword	NPC Non-player character Cell membrane Lysosome Lipid storage disorder Cholesterol Lipid Protein NPC1 Gene Irving Langmuir Langmuir Sphingomyelin Mutation Niemann\u2013Pick disease Digital rights management Endosome DPPC Annexin A6 Dipalmitoylphosphatidylcholine
Content Type	Text
Resource Type	Article
Subject	Molecular Biology Biotechnology

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Impaired dynamics of the late endosome/lysosome compartment in human Niemann–Pick type C skin fibroblasts carrying mutation in NPC1 gene

Annexin A6 involvement in the organization of cholesterol-rich membrane microdomains : evidence from cells of the Niemann-Pick type C disease patients and biomimetic lipid monolayers

Cholesterol Overload: Contact Sites to the Rescue!

Selective Degradation Permits a Feedback Loop Controlling Annexin A6 and Cholesterol Levels in Endolysosomes of NPC1 Mutant Cells

NPC intracellular cholesterol transporter 1 (NPC1)-mediated cholesterol export from lysosomes

Arf6-mediated endosome recycling reverses lipid accumulation defects in niemann-pick type c disease (2008).

The role of palmitic acid in pulmonary surfactant systems by Langmuir monolayer study: Lipid–peptide interactions

Evidence for annexin A6-dependent plasma membrane remodelling of lipid domains

Annexin A6 and Late Endosomal Cholesterol Modulate Integrin Recycling and Cell Migration

Interaction of AnxA6 with isolated and artificial lipid microdomains; importance of lipid composition and calcium content

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