GPIHBP1 and the processing of triglyceride-rich lipoproteins.
GPIHBP1 and the processing of triglyceride-rich lipoproteins.
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DOI：
10.2217/clp.10.43
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发表时间：
2010-08-01
影响因子：
--
通讯作者：
Beigneux AP
中科院分区：
文献类型：
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作者：
Beigneux AP
GPIHBP1是对富含甘油三酯的脂蛋白进行脂解所需的一组蛋白质的新添加。GPIHBP1含有一个酸性结构域和一个含有10个半胱氨酸的Ly6结构域。GPIHBP1与脂蛋白脂酶(LPL)强烈结合，可能将LPL系在毛细血管的管腔表面。小鼠的Gpihbp1失活与乳清血浆和严重的乳糜粒微粒血症有关，即使在低脂肪饮食中也是如此。最近，在患有严重乳糜体微粒血症的人中发现了四个GPIHBP1错义突变(C65Y、C65S、C68G和Q115P)。所有四个突变都涉及GPIHBP1的S Ly6结构域中的高度保守残基。本文将对GPIHBP1的S在乳糜粒加工过程中的作用和乳糜粒微粒症的发病机制的研究进展进行综述。
GPIHBP1 is a new addition to a group of proteins required for the lipolysis of triglyceride-rich lipoproteins. GPIHBP1 contains an acidic domain and an Ly6 domain with ten cysteines. GPIHBP1 binds lipoprotein lipase (LPL) avidly and likely tethers LPL to the luminal surface of capillaries. Inactivation of Gpihbp1 in mice is associated with milky plasma and severe chylomicronemia, even on a low-fat chow diet. Recently, four missense mutations in GPIHBP1 were identified in humans with severe chylomicronemia (C65Y, C65S, C68G, and Q115P). All four mutations involve highly conserved residues within GPIHBP1’s Ly6 domain. This review will provide an update on GPIHBP1’s role in the processing of chylomicrons and the pathogenesis of chylomicronemia.