Document Detail


Glycosylphosphatidylinositol-anchored high-density lipoprotein-binding protein 1 and the intravascular processing of triglyceride-rich lipoproteins.
MedLine Citation:
PMID:  23020258     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Lipoprotein lipase (LPL) is produced by parenchymal cells, mainly adipocytes and myocytes, but is involved in hydrolysing triglycerides in plasma lipoproteins at the capillary lumen. For decades, the mechanism by which LPL reaches its site of action in capillaries was unclear, but this mystery was recently solved. Glycosylphosphatidylinositol-anchored high-density lipoprotein-binding protein 1 (GPIHBP1), a glycosylphosphatidylinositol-anchored protein of capillary endothelial cells, 'picks up' LPL from the interstitial spaces and shuttles it across endothelial cells to the capillary lumen. When GPIHBP1 is absent, LPL is mislocalized to the interstitial spaces, leading to severe hypertriglyceridaemia. Some cases of hypertriglyceridaemia in humans are caused by GPIHBP1 mutations that interfere with the ability of GPIHBP1 to bind to LPL, and some are caused by LPL mutations that impair the ability of LPL to bind to GPIHBP1. Here, we review recent progress in understanding the role of GPIHBP1 in health and disease and discuss some of the remaining unresolved issues regarding the processing of triglyceride-rich lipoproteins.
Authors:
O Adeyo; C N Goulbourne; A Bensadoun; A P Beigneux; L G Fong; S G Young
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review     Date:  2012-11-01
Journal Detail:
Title:  Journal of internal medicine     Volume:  272     ISSN:  1365-2796     ISO Abbreviation:  J. Intern. Med.     Publication Date:  2012 Dec 
Date Detail:
Created Date:  2012-11-21     Completed Date:  2013-02-14     Revised Date:  2014-03-09    
Medline Journal Info:
Nlm Unique ID:  8904841     Medline TA:  J Intern Med     Country:  England    
Other Details:
Languages:  eng     Pagination:  528-40     Citation Subset:  IM    
Copyright Information:
© 2012 The Association for the Publication of the Journal of Internal Medicine.
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MeSH Terms
Descriptor/Qualifier:
Animals
Capillaries / metabolism
Carrier Proteins* / genetics,  metabolism
Chylomicrons / metabolism
Endothelial Cells / physiology*
Endothelium, Vascular / physiology
Genetic Predisposition to Disease
Humans
Hypertriglyceridemia* / genetics,  metabolism
Lipolysis / genetics
Lipoprotein Lipase* / genetics,  metabolism
Mice
Mutation, Missense
Protein Transport / genetics
Receptors, Lipoprotein* / genetics,  metabolism
Triglycerides / metabolism
Grant Support
ID/Acronym/Agency:
P01 HL090553/HL/NHLBI NIH HHS; P01 HL090553/HL/NHLBI NIH HHS; R01 HL087228/HL/NHLBI NIH HHS; R01 HL087228/HL/NHLBI NIH HHS; R01 HL094732/HL/NHLBI NIH HHS
Chemical
Reg. No./Substance:
0/Carrier Proteins; 0/Chylomicrons; 0/GPI-HBP1 protein, mouse; 0/GPIHBP1 protein, human; 0/Receptors, Lipoprotein; 0/Triglycerides; EC 3.1.1.34/Lipoprotein Lipase
Comments/Corrections

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