ESI-MS quantitation of increased sphingomyelin in Niemann-Pick disease type B HDL.

Abstract:

:HDLs have been proposed to have antiatherogenic properties because of their role in reverse cholesterol transport as lipid acceptors. To elucidate the phospholipid profile of these particles, we used electrospray ionization mass spectrometry to examine the phosphatidylcholine (PC) and sphingomyelin (SM) composition of HDLs purified from plasma and nascently generated in vitro from fibroblasts. We also quantitatively compared the phospholipids present in these lipoproteins between normal and Niemann-Pick disease type B (NPD-B) subjects characterized by sphingomyelinase (SMase) deficiency. We demonstrated that plasma HDLs from NPD-B were significantly enriched in SM by an average of 28%, particularly the palmitoyl SM (with an increase of 95%), which accounted for approximately 25-44% of total SM molecular species. Similarly, we observed an increase of approximately 63% in total SM levels in nascent HDLs prepared from NPD-B fibroblasts. Although PC levels in nascent HDLs were comparable between control and NPD-B cells, there was a 95% increase in total PC levels similar to that of SM in plasma HDLs extracted from NPD-B subjects. These data provide insight into the structure of HDLs and identify potential new roles for SMase in lipoprotein metabolism.

journal_name

J Lipid Res

authors

Lee CY,Lesimple A,Larsen A,Mamer O,Genest J

doi

10.1194/jlr.M500011-JLR200

keywords:

subject

Has Abstract

pub_date

2005-06-01 00:00:00

pages

1213-28

issue

6

eissn

0022-2275

issn

1539-7262

pii

M500011-JLR200

journal_volume

46

pub_type

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