ALP/Enigma PDZ-LIM domain proteins in the heart.
ALP/Enigma PDZ-LIM domain proteins in the heart.
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DOI：
10.1093/jmcb/mjp038
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发表时间：
2010-04
影响因子：
5.5
通讯作者：
M. Zheng;Hongqiang Cheng;Indroneal Banerjee;Ju Chen
中科院分区：
文献类型：
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作者：
M. Zheng;Hongqiang Cheng;Indroneal Banerjee;Ju Chen
肌动蛋白相关LIM蛋白(ALP)和迷宫蛋白(Enigma)是突触后密度95、大圆盘和闭锁带-1(PDZ)-LIN-11、Isl1和Mec-3(LIM)结构域的两个亚家族。ALP家族成员有一个PDZ和一个LIM结构域，而Enigma蛋白则包含一个PDZ和三个LIM结构域。已在哺乳动物中鉴定出四种ALP和三种Enigma蛋白，每种蛋白都有多种剪接变体和独特的表达模式。在功能上，这些蛋白质通过它们的PDZ结构域与α-肌动蛋白结合，并通过它们的LIM结构域或其他内部蛋白质相互作用结构域与其他蛋白质结合，包括信号分子。ALP和Enigma蛋白与心肌和骨骼肌结构、功能和疾病、神经功能、双相情感障碍、肿瘤生长、血小板和上皮细胞运动以及骨形成有关。本文综述了ALP/Enigma PDZ-Lim结构域蛋白在心肌中生物学作用的最新进展，并对这些蛋白的突变与人类心脏病相关的机制提供了深入的认识。
Actinin-associated LIM protein (ALP) and Enigma are two subfamilies of Postsynaptic density 95, discs large and zonula occludens-1 (PDZ)-Lin-11, Isl1 and Mec-3 (LIM) domain containing proteins. ALP family members have one PDZ and one LIM domain, whereas Enigma proteins contain one PDZ and three LIM domains. Four ALP and three Enigma proteins have been identified in mammals, each having multiple splice variants and unique expression patterns. Functionally, these proteins bind through their PDZ domains to alpha-actinin and bind through their LIM domains or other internal protein interaction domains to other proteins, including signaling molecules. ALP and Enigma proteins have been implicated in cardiac and skeletal muscle structure, function and disease, neuronal function, bipolar disorder, tumor growth, platelet and epithelial cell motility and bone formation. This review will focus on recent advances in the biological roles of ALP/Enigma PDZ-LIM domain proteins in cardiac muscle and provide insights into mechanisms by which mutations in these proteins are related to human cardiac disease.