Analysis of the three-dimensional distributions of alpha-actinin, ankyrin, and filamin in developing hearts of normal and cardiac mutant axolotls (Ambystoma mexicanum).
Analysis of the three-dimensional distributions of alpha-actinin, ankyrin, and filamin in developing hearts of normal and cardiac mutant axolotls (Ambystoma mexicanum).
复制标题
分析正常和心脏突变蝾螈（Ambystoma mexicanum）发育中心脏中α-肌动蛋白、锚蛋白和纤丝蛋白的三维分布。
DOI：
10.1007/s004290050034
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发表时间：
1997
期刊：
影响因子：
--
通讯作者：
Lemanski,LF
中科院分区：
文献类型：
--
作者：
Lemanski,SF;Kovacs,CP;Lemanski,LF
α-辅肌动蛋白是一种肌动蛋白结合蛋白，有助于质膜的稳定，并有助于将细胞器固定在各种细胞类型中的位置。在肌肉中，它是有组织肌原纤维Z线的主要组成部分。锚蛋白结合细胞骨架系统的各种元件，包括微管、微丝和中间丝，并可帮助将这些结构锚于细胞膜。细丝蛋白是一种先从鸡平滑肌中分离得到的肌动蛋白相关蛋白。此外，细丝蛋白是一种形成凝胶的蛋白质，有助于形成松散但厚的肌动蛋白细丝网络。这些蛋白质与其他细胞骨架蛋白一起工作，以允许脊椎动物心肌细胞的收缩。在一个独特的菌株的蝾螈（Ambystoma mexicanum）一个简单的隐性突变，指定的基因，结果在一个不完全分化的心脏受影响的胚胎。虽然突变体（c/c）胚胎形成心脏，但由于组织化的肌节肌原纤维形成失败，它们不跳动。本研究旨在检测从早期心跳阶段37到晚期胚胎阶段42的正常和突变心脏中肌原纤维形成过程中三种不同收缩细胞骨架蛋白（α-辅肌动蛋白、锚蛋白和细丝蛋白）的三维分布。我们的研究结果表明，随着发育的进展，收缩蛋白在正常心脏中变得越来越好。在突变的心脏中，尽管蛋白质以几乎正常的量存在，但它们不能形成正常组织的肌原纤维。
α-Actinin is an actin binding protein that assists in the stabilization of the plasma membrane and helps to fix organelles in position in a variety of cell types. In muscle, it is a major component of the Z-lines of organized myofibrils. Ankyrin binds to various elements of the cytoskeletal system including microtubules, microfilaments, and intermediate filaments and may help to anchor these structures to the cell membrane. Filamin is a well-characterized actin-associated protein first isolated from chicken smooth muscle. In addition, filamin is a gel-forming protein which aids in the formation of a loose, yet thick, network of actin filaments. These proteins work together, in conjunction with other cytoskeletal proteins, to permit the contractions of heart muscle cells in vertebrates. In a unique strain of the axolotls (Ambystoma mexicanum) a simple recessive mutation, designated by genec, results in an incomplete differentiation of the hearts of affected embryos. Although the mutant (c/c) embryos form hearts, they do not beat because of a failure in the formation of organized sarcomeric myofibrils. The current study was undertaken to examine the three-dimensional distributions of three different contractile-cytoskeletal proteins (α-actinin, ankyrin, and filamin) during myofibrillogenesis in normal and mutant hearts from early heart-beat stage 37 through advanced embryonic stage 42. Our results demonstrate that the contractile proteins become increasingly better organized in normal hearts as development progresses. In mutant hearts, although the proteins are present in almost normal amounts, they fail to form normally organized myofibrils.