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Epidermal cell junctions and their regulation by p63 in health and disease

机译：表皮细胞连接及其在健康和疾病中的p63调控

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摘要

As the outermost tissue of the body, the epidermis is the first physical barrier for any pressure, stress or trauma. Several specialized cell-matrix and cell-cell adhesion structures, together with an intracellular network of dedicated intermediate filaments, are required to confer critical resilience to mechanical stress. The transcription factor p63 is a master regulator of gene expression in the epidermis and in other stratified epithelia. It has been extensively demonstrated that p63 positively controls a large number of tissue-specific genes, including those encoding a large fraction of tissue-restricted cell adhesion molecules. Consistent with p63 functions in cell adhesion and in epidermal differentiation, heterozygous mutations clustered mainly in the p63 C-terminus are causative of AEC syndrome, an autosomal dominant disorder characterized by cleft palate, ankyloblepharon and ectodermal dysplasia associated with severe skin erosions, bleeding and infections. The molecular basis of skin erosions in AEC patients is not fully understood, although defects in desmosomes and in other cell junctions are likely to be involved. Here, we provide an extensive review of the different epidermal cell junctions that cooperate to withstand mechanical stress and on the mechanisms by which p63 regulates gene expression of their components in healthy skin and in AEC syndrome. Collectively, advancement in understanding the molecular mechanisms by which epidermal cell junctions precisely exert their functions and how p63 orchestrates their coordinated expression, will ultimately lead to insight into developing future strategies for the treatment of AEC syndrome and more in generally for diseases that share an overlapping phenotype.
机译：作为人体的最外层组织，表皮是任何压力，压力或创伤的第一个物理屏障。需要几种专门的细胞基质和细胞间粘附结构，以及专用中间丝的胞内网络，以赋予对机械应力的临界弹性。转录因子p63是表皮和其他分层上皮细胞中基因表达的主要调控因子。已广泛证明p63阳性控制大量组织特异性基因，包括编码大部分组织受限细胞粘附分子的基因。与p63在细胞粘附和表皮分化中的功能一致，主要在p63 C端聚集的杂合突变是AEC综合征的病因，AEC综合征是一种常染色体显性遗传疾病，其特征为left裂，上颌甲龙和外胚层发育异常，伴有严重的皮肤糜烂，出血和感染。 。尽管可能涉及桥粒和其他细胞接头的缺陷，但尚未完全了解AEC患者皮肤糜烂的分子基础。在这里，我们提供了对不同的表皮细胞连接进行合作的广泛综述，这些连接可以承受机械应力，并探讨了p63调节健康皮肤和AEC综合征中其成分的基因表达的机制。总的来说，对表皮细胞连接精确发挥其功能的分子机制以及p63如何协调其协同表达的分子机制的了解，将最终导致对开发治疗AEC综合征的未来策略以及更普遍地针对共有重叠疾病的策略的见解。表型。

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