磷脂酰肌醇转移蛋白及其同源物的哺乳动物疾病
Mammalian diseases of phosphatidylinositol transfer proteins and their homologs.
作者信息
Nile Aaron H, Bankaitis Vytas A, Grabon Aby
机构信息
Department of Cell & Developmental Biology, Lineberger Comprehensive Cancer Center School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599-27090, USA.
出版信息
Clin Lipidol. 2010 Dec 1;5(6):867-897. doi: 10.2217/clp.10.67.
Abstract
Inositol and phosphoinositide signaling pathways represent major regulatory systems in eukaryotes. The physiological importance of these pathways is amply demonstrated by the variety of diseases that involve derangements in individual steps in inositide and phosphoinositide production and degradation. These diseases include numerous cancers, lipodystrophies and neurological syndromes. Phosphatidylinositol transfer proteins (PITPs) are emerging as fascinating regulators of phosphoinositide metabolism. Recent advances identify PITPs (and PITP-like proteins) to be coincidence detectors, which spatially and temporally coordinate the activities of diverse aspects of the cellular lipid metabolome with phosphoinositide signaling. These insights are providing new ideas regarding mechanisms of inherited mammalian diseases associated with derangements in the activities of PITPs and PITP-like proteins.
摘要
肌醇和磷酸肌醇信号通路是真核生物中的主要调节系统。这些通路的生理重要性通过多种疾病得到了充分证明，这些疾病涉及肌醇磷脂和磷酸肌醇生成与降解的各个步骤紊乱。这些疾病包括许多癌症、脂肪营养不良和神经综合征。磷脂酰肌醇转移蛋白（PITPs）正成为磷酸肌醇代谢中引人入胜的调节因子。最近的进展表明，PITPs（以及类PITP蛋白）是巧合探测器，它们在空间和时间上协调细胞脂质代谢组的各个方面与磷酸肌醇信号的活动。这些见解为与PITPs和类PITP蛋白活性紊乱相关的遗传性哺乳动物疾病的机制提供了新的思路。
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