Department of Physiology, Johns Hopkins School of Medicine, Baltimore, MD 21205-2185, USA.
Trends Biochem Sci. 2012 Jan;37(1):32-41. doi: 10.1016/j.tibs.2011.09.003. Epub 2011 Oct 17.
Cardiolipin, the signature phospholipid of mitochondria, is a lipid dimer that is important for a diverse range of mitochondrial activities beyond the process of ATP production. Thus not surprisingly, derangements in cardiolipin metabolism are now appreciated to contribute to an assortment of pathological conditions. A comprehensive inventory of enzymes involved in cardiolipin biosynthesis and remodeling was just recently obtained. Post-biosynthesis, the acyl chain composition of cardiolipin is modified by up to three distinct remodeling enzymes that produce either a homogeneous tissue-specific mature form of cardiolipin or alternatively 'bad' cardiolipin that has been linked to mitochondrial dysfunction. In this review, we initially focus on the newly identified players in cardiolipin metabolism and then shift our attention to how changes in cardiolipin metabolism contribute to human disease.
心磷脂是线粒体的标志性磷脂，是一种脂质二聚体，除了参与 ATP 生成过程外，在心磷脂对于多种线粒体活动中也起着重要作用。因此，毫不奇怪，现在人们已经认识到心磷脂代谢的紊乱会导致多种病理状况。最近刚刚获得了涉及心磷脂生物合成和重塑的酶的综合清单。生物合成后，心磷脂的酰基链组成通过多达三种不同的重塑酶进行修饰，这些酶产生均质的组织特异性成熟形式的心磷脂，或者是已经与线粒体功能障碍相关的“坏”心磷脂。在这篇综述中，我们最初关注心磷脂代谢中的新鉴定出的参与者，然后将注意力转移到心磷脂代谢的变化如何导致人类疾病。
