Wenzel Jürgen J, Piehler Armin, Kaminski Wolfgang E
Institut für Klinische Chemie und Laboratoriumsmedizin, Johannes Gutenberg-Universität, Mainz.
Med Klin (Munich). 2007 Jul 15;102(7):524-30. doi: 10.1007/s00063-007-1066-8.
The controlled uptake and release of lipid compounds is a hallmark feature of living cells. Numerous factors have been implicated in these complex transmembrane transport processes. The recent discovery of a novel class of transporter molecules, the group of A-subclass ATP-binding cassette (ABC) transporters, has brought new insights into the molecular basis of cellular lipid transport. Available evidence indicates that individual ABC A-subclass transporters function as key components of highly specialized cellular lipid export machineries in major physiological systems and, when defective, cause hereditary diseases in the cardiovascular, respiratory, visual and integumentary systems, respectively. Intriguingly, a steadily growing body of evidence suggests that ABC A lipid transporters play important roles in the pathogenesis of complex disorders with high incidence including atherosclerosis, Alzheimer's disease and age-related macula degeneration. The present article reviews the biology of this emerging group of proteins and their implication in human pathologies.
脂质化合物的可控摄取和释放是活细胞的一个标志性特征。许多因素都与这些复杂的跨膜运输过程有关。最近发现了一类新型转运分子，即A亚类ATP结合盒（ABC）转运蛋白家族，这为细胞脂质运输的分子基础带来了新的见解。现有证据表明，单个ABC A亚类转运蛋白在主要生理系统中作为高度专业化的细胞脂质输出机制的关键组成部分发挥作用，并且当出现缺陷时，分别会导致心血管、呼吸、视觉和皮肤系统的遗传性疾病。有趣的是，越来越多的证据表明，ABC A脂质转运蛋白在包括动脉粥样硬化、阿尔茨海默病和年龄相关性黄斑变性在内的高发性复杂疾病的发病机制中发挥重要作用。本文综述了这一新兴蛋白质群体的生物学特性及其在人类病理学中的意义。
