Targeted mutation of plakoglobin in mice reveals essential functions of desmosomes in the embryonic heart
Targeted mutation of plakoglobin in mice reveals essential functions of desmosomes in the embryonic heart
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DOI：
10.1083/jcb.135.1.215
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发表时间：
1996-10-01
影响因子：
7.8
通讯作者：
Birchmeier, W
中科院分区：
文献类型：
--
作者：
Ruiz, P;Brinkmann, V;Birchmeier, W
斑珠蛋白（γ-连环蛋白）是蛋白质的犰狳家族的成员，是桥粒的细胞质斑以及其他粘附细胞连接的组成部分，并且参与细胞骨架丝与特异性钙粘蛋白的锚定。我们已经在小鼠中产生了斑珠蛋白基因的无效突变。纯合-/-突变动物在胚胎发育的第12-16天由于心脏功能缺陷而死亡。通常情况下，心室破裂，血液淹没心包。这种组织不稳定性与心脏中桥粒的缺乏有关，但与上皮器官中的桥粒无关。相反，在心脏中形成延伸的粘附连接，其含有桥粒蛋白，即，桥粒斑蛋白因此，斑珠蛋白是心肌桥粒的一个重要组成部分，似乎发挥了至关重要的作用，在分选出桥粒和粘附连接组件，因此在闰盘的架构和心脏组织的稳定。
Plakoglobin (gamma-catenin), a member of the armadillo family of proteins, is a constituent of the cytoplasmic plaque of desmosomes, as well as of other adhering cell junctions, and is involved in anchorage of cytoskeletal filaments to specific cadherins. We have generated a null mutation of the plakoglobin gene in mice. Homozygous -/- mutant animals die between days 12-16 of embryogenesis due to defects in heart function. Often, heart ventricles burst and blood floods the pericard. This tissue instability correlates with the absence of desmosomes in heart, but not in epithelial organs. Instead, extended adherens junctions are formed in the heart, which contain desmosomal proteins, i.e., desmoplakin. Thus, plakoglobin is an essential component of myocardiac desmosomes and seems to play a crucial role in the sorting out of desmosomal and adherens junction components, and consequently in the architecture of intercalated discs and the stabilization of heart tissue.