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The keratin-desmosome scaffold of internal epithelia in health and disease – The plot is thickening
Current Opinion in Cell Biology ( IF 7.5 ) Pub Date : 2023-11-24 , DOI: 10.1016/j.ceb.2023.102282
Diana M Toivola 1 , Lauri Polari 1 , Tobias Schwerd 2 , Nicolas Schlegel 3 , Pavel Strnad 4
Affiliation  

Keratin (K) intermediate filaments are attached to desmosomes and constitute the orchestrators of epithelial cell and tissue architecture. While their relevance in the epidermis is well recognized, our review focuses on their emerging importance in internal epithelia. The significance of keratin-desmosome scaffolds (KDSs) in the intestine is highlighted by transgenic mouse models and individuals with inflammatory bowel disease who display profound KDS alterations. In lung, high K8 expression defines a transitional cell subset during regeneration, and K8 variants are associated with idiopathic pulmonary fibrosis. Inherited variants in desmosomal proteins are overrepresented in idiopathic lung fibrosis, and familiar eosinophilic esophagitis. K18 serum fragments are established hepatocellular injury markers that correlate with the extent of histological inflammation. K17 expression is modified in multiple tumors, and K17 levels might be of prognostic relevance. These data should spur further studies on biological roles of these versatile tissue protectors and efforts on their therapeutic targeting.



中文翻译:

健康和疾病中内上皮的角蛋白-桥粒支架——情节正在变厚

角蛋白 (K) 中间丝附着在桥粒上,构成上皮细胞和组织结构的协调器。虽然它们在表皮中的相关性已得到广泛认可,但我们的综述重点关注它们在内部上皮中的新兴重要性。转基因小鼠模型和患有炎症性肠病的个体表现出严重的 KDS 改变,强调了角蛋白桥粒支架 (KDS) 在肠道中的重要性。在肺中,高 K8 表达定义了再生过程中的过渡细胞亚群,并且 K8 变异与特发性肺纤维化相关。桥粒蛋白的遗传性变异在特发性肺纤维化和熟悉的嗜酸性粒细胞性食管炎中过多出现。K18 血清碎片是已建立的肝细胞损伤标记物,与组织学炎症的程度相关。K17 表达在多种肿瘤中发生改变,K17 水平可能与预后相关。这些数据应该会促进对这些多功能组织保护剂的生物学作用的进一步研究以及对其治疗靶向的努力。

更新日期:2023-11-26
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