King Abdullah International Medical Research Center, King Saud bin Abdulaziz University Health Sciences, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Al Ahsa, Saudi Arabia.
John P. Stevens High School, North Edison, NJ, USA.
Adv Exp Med Biol. 2020;1276:37-52. doi: 10.1007/978-981-15-6082-8_4.
Microsomal triglyceride transfer protein (MTP) was first identified as an endoplasmic reticulum (ER) resident protein that helps in the transfer of neutral lipids to nascent apolipoprotein B (apoB). Its critical role in the assembly and secretion of apoB-containing lipoproteins was identified in abetalipoproteinemia patients who have mutations in MTP and completely lack apoB-containing lipoproteins in the circulation. It has been established now that MTP not only is involved in the transfer of neutral lipids but also plays a role in cholesterol ester and cluster of differentiation 1d (CD1d) biosynthesis. Besides neutral lipids, MTP may also help in the transfer of sphingolipids such as ceramides and sphingomyelin to the apoB-containing lipoproteins. MTP is a multifunctional protein, and its deregulation during pathophysiological conditions gives rise to different metabolic conditions. This book chapter discusses the physiological role and regulation of MTP to maintain the homeostasis of lipids and lipoproteins. It also reviews the regulation of MTP during certain pathophysiological conditions and provides a brief overview of therapeutic interventions that can be possibly used to target its activity or expression to alleviate some of these metabolic diseases.
微粒体甘油三酯转移蛋白（MTP）最初被鉴定为内质网（ER）驻留蛋白，有助于将中性脂质转移到新生载脂蛋白 B（apoB）。在载脂蛋白 B 缺失脂蛋白血症患者中发现了其在载脂蛋白 B 含脂蛋白组装和分泌中的关键作用，这些患者的 MTP 发生突变，完全缺乏循环中的载脂蛋白 B 含脂蛋白。现在已经确定，MTP 不仅参与中性脂质的转移，而且还参与胆固醇酯和分化簇 1d（CD1d）的生物合成。除了中性脂质外，MTP 还可能有助于将神经酰胺和鞘磷脂等鞘脂转移到载脂蛋白 B 含脂蛋白中。MTP 是一种多功能蛋白，其在病理生理条件下的失调会导致不同的代谢状况。本章讨论了 MTP 的生理作用和调节，以维持脂质和脂蛋白的体内平衡。它还回顾了 MTP 在某些病理生理条件下的调节，并简要概述了可能用于靶向其活性或表达以减轻其中一些代谢疾病的治疗干预措施。
