Plakophilins, desmogleins, and pemphigus: the tail wagging the dog.

Plakophilins, desmogleins, and pemphigus: the tail wagging the dog.
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DOI:
10.1038/jid.2013.491
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发表时间:
2014-04
影响因子:
6.5
通讯作者:
Payne, Aimee S.
Payne, Aimee S.
中科院分区:
医学1区
文献类型:
--
作者:
Ellebrecht, Christoph T.;Payne, Aimee S.

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自身免疫性疾病寻常天疱疮(PV)证明了桥粒细胞粘附对人类健康的重要性,其中针对桥粒钙粘蛋白桥粒芯蛋白3的细胞外结构域的自身抗体引起皮肤和粘膜的潜在致命性起泡。Tucker等人描述了桥粒细胞质斑块蛋白(plakophilin-1)的表达增强如何通过诱导钙非依赖性超粘附桥粒来保护角质形成细胞免受PV IgG诱导的细胞粘附丧失。这项研究完美地证明了桥粒粘附可以通过桥粒芯蛋白尾部的分子相互作用来调节,并表明这些新的调节途径可能被用于治疗人类疾病。
The importance of desmosomal cell adhesion to human health is evidenced by the autoimmune disease pemphigus vulgaris (PV), in which autoantibodies against the extracellular domain of the desmosomal cadherin desmoglein 3 cause potentially fatal blistering of the skin and mucous membranes. Tucker et al. describe how enhanced expression of a desmosomal cytoplasmic plaque protein, plakophilin-1, protects keratinocytes from PV IgG-induced loss of cell adhesion by inducing calcium-independent hyperadhesive desmosomes. This study beautifully demonstrates that desmosomal adhesion can be modulated by the molecular interactions of the desmoglein tail and suggests that these novel regulatory pathways may possibly be exploited in treating human disease.
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