The desmosome and pemphigus
The desmosome and pemphigus
复制标题
桥粒和天疱疮
DOI：
--
复制
发表时间：
2008
影响因子：
2.3
通讯作者：
J. Waschke
中科院分区：
文献类型：
--
作者：
J. Waschke
桥粒是片状细胞间粘附连接（“粘附斑”），其与相关的粘附连接一起为细胞间粘附提供机械强度。因此，桥粒在经受显著机械应力的组织如复层上皮和心肌中丰富并不奇怪。桥粒粘附是基于钙粘蛋白型粘附分子的Ca 2+依赖性、同嗜性和异嗜性相互作用。桥粒钙粘蛋白通过犰狳和斑蛋白家族的衔接蛋白锚定在中间丝细胞骨架上。桥粒是受调控的动态结构，因此是信号传导途径的靶点，信号传导途径控制桥粒的分子组成和粘附特性。此外，有证据表明桥粒成分本身在生理和病理条件下参与由外向内的信号传导。桥粒粘附紊乱导致了许多疾病的发病机制，如天疱疮，这是由针对桥粒钙粘蛋白的自身抗体引起的。除了天疱疮，桥粒相关疾病是由其他机制，如遗传缺陷或细菌毒素引起的。由于这些疾病中的大多数影响皮肤，桥粒不仅对受到其复杂结构和分子组成启发的细胞生物学家感兴趣，而且对面临严重起泡皮肤病（如天疱疮）患者的临床医生也很感兴趣。为了开发疾病特异性治疗方法，需要对桥粒的分子组成和调节有更多的了解。
Desmosomes are patch-like intercellular adhering junctions (“maculae adherentes”), which, in concert with the related adherens junctions, provide the mechanical strength to intercellular adhesion. Therefore, it is not surprising that desmosomes are abundant in tissues subjected to significant mechanical stress such as stratified epithelia and myocardium. Desmosomal adhesion is based on the Ca2+-dependent, homo- and heterophilic transinteraction of cadherin-type adhesion molecules. Desmosomal cadherins are anchored to the intermediate filament cytoskeleton by adaptor proteins of the armadillo and plakin families. Desmosomes are dynamic structures subjected to regulation and are therefore targets of signalling pathways, which control their molecular composition and adhesive properties. Moreover, evidence is emerging that desmosomal components themselves take part in outside-in signalling under physiologic and pathologic conditions. Disturbed desmosomal adhesion contributes to the pathogenesis of a number of diseases such as pemphigus, which is caused by autoantibodies against desmosomal cadherins. Beside pemphigus, desmosome-associated diseases are caused by other mechanisms such as genetic defects or bacterial toxins. Because most of these diseases affect the skin, desmosomes are interesting not only for cell biologists who are inspired by their complex structure and molecular composition, but also for clinical physicians who are confronted with patients suffering from severe blistering skin diseases such as pemphigus. To develop disease-specific therapeutic approaches, more insights into the molecular composition and regulation of desmosomes are required.
登录
查看更多内容
桥粒钙粘蛋白 Dsg1 的细胞质结构域与斑球蛋白的相互作用。
DOI：
--
发表时间：
1994
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
Mathur,M;Goodwin,L;Cowin,P
通讯作者：
Cowin,P
影响因子：
6.5
作者：
Dusek, Rachel L.;Godsel, Lisa M.;Green, Kathleen J.
通讯作者：
Green, Kathleen J.
影响因子：
4
作者：
P. J. Koch;M. Mahoney;G. Cotsarelis;K. Rothenberger;R. Lavker;J. Stanley
通讯作者：
P. J. Koch;M. Mahoney;G. Cotsarelis;K. Rothenberger;R. Lavker;J. Stanley
影响因子：
20.1
作者：
Angst, BD;Khan, LUR;Gourdie, RG
通讯作者：
Gourdie, RG
斑珠蛋白的桥粒钙粘蛋白结合域。
DOI：
10.1074/jbc.271.18.10904
发表时间：
1996
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
Witcher,LL;Collins,R;Puttagunta,S;Mechanic,SE;Munson,M;Gumbiner,B;Cowin,P
通讯作者：
Cowin,P