GPIHBP1 and Plasma Triglyceride Metabolism

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• 作者：Loren G. Fong¹ ; ^{lfong@mednet.ucla.edu" class="auth_mail" title="E-mail the corresponding author} ; Stephen G. Young¹ ; ² ; ^{sgyoung@mednet.ucla.edu" class="auth_mail" title="E-mail the corresponding author} ; Anne P. Beigneux¹ ; André ; Bensadoun³ ; Monika Oberer⁴ ; Haibo Jiang⁵ ; Michael Ploug⁶ ; ⁷ ; ^{m-ploug@finsenlab.dk" class="auth_mail" title="E-mail the corresponding author}
• 关键词：hypertriglyceridemia ; chylomicronemia ; lipoprotein lipase ; lipid transport ; endothelial cells ; LU (Ly6/uPAR) protein family
• 刊名：Trends in Endocrinology & Metabolism
• 出版年：2016
• 出版时间：July 2016
• 年：2016
• 卷：27
• 期：7
• 页码：455-469
• 全文大小：4109 K

文摘

GPIHBP1, a GPI-anchored protein in capillary endothelial cells, is crucial for the lipolytic processing of triglyceride-rich lipoproteins (TRLs). GPIHBP1 shuttles lipoprotein lipase (LPL) to its site of action in the capillary lumen and is essential for the margination of TRLs along capillaries – such that lipolytic processing can proceed. GPIHBP1 also reduces the unfolding of the LPL catalytic domain, thereby stabilizing LPL catalytic activity. Many different GPIHBP1 mutations have been identified in patients with severe hypertriglyceridemia (chylomicronemia), the majority of which interfere with folding of the protein and abolish its capacity to bind and transport LPL. The discovery of GPIHBP1 has substantially revised our understanding of intravascular triglyceride metabolism but has also raised many new questions for future research.