<br>组织蛋白酶 C 在健康和疾病中的作用：从结构见解到治疗前景,Journal of Translational Medicine

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组织蛋白酶 C 在健康和疾病中的作用：从结构见解到治疗前景
Journal of Translational Medicine ( IF 6.1 ) Pub Date : 2024-08-20 , DOI: 10.1186/s12967-024-05589-7
Chakriya Chitsamankhun ₁ , Nutwara Siritongtaworn ₁ , B P J Fournier ₂ , Kanokwan Sriwattanapong ₁ , Thanakorn Theerapanon ₁ , Lakshman Samaranayake _{3,

4} , Thantrira Porntaveetus _{1,

5}

Affiliation

组织蛋白酶 C (CTSC) 是一种溶酶体半胱氨酸蛋白酶，在肺、肾、肝和脾中高水平表达。它在细胞毒性 T 细胞、自然杀伤细胞（颗粒酶 A 和 B）、肥大细胞（糜酶和类胰蛋白酶）和中性粒细胞（组织蛋白酶 G、中性粒细胞弹性蛋白酶、蛋白酶 3）中丝氨酸蛋白酶的激活中发挥关键作用，强调了其在免疫和炎症防御。在这里，我们全面回顾了 CTSC 的结构属性、合成和功能，重点关注与中性粒细胞丝氨酸蛋白酶相关的几种综合征的病因病理学相关的其变体，包括 Papillon-Lefevre 综合征 (PLS)、Haim-Munk 综合征 (HMS) ）和侵袭性牙周炎（AP）。这些综合征的特征是掌跖角化过度和早发性牙周炎（严重牙龈疾病），导致牙齿过早脱落。由于 CTSC 在这些疾病和其他几种疾病中发挥的关键作用，它正在被探索作为自身免疫性和炎症性疾病的潜在治疗靶点。该综述还深入讨论了 CTSC 的基因变异，特别是它们与慢性阻塞性肺病 (COPD)、COVID-19、各种癌症、抗中性粒细胞胞浆抗体 (ANCA) 相关血管炎、心源性猝死的假定关联。 SCD）、动脉粥样硬化性血管疾病和神经炎症性疾病。最后，讨论了 CTSC 在一系列人类疾病中的治疗潜力。

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Cathepsin C in health and disease: from structural insights to therapeutic prospects

Cathepsin C (CTSC) is a lysosomal cysteine protease constitutively expressed at high levels in the lung, kidney, liver, and spleen. It plays a key role in the activation of serine proteases in cytotoxic T cells, natural killer cells (granzymes A and B), mast cells (chymase and tryptase) and neutrophils (cathepsin G, neutrophil elastase, proteinase 3) underscoring its pivotal significance in immune and inflammatory defenses. Here, we comprehensively review the structural attributes, synthesis, and function of CTSC, with a focus on its variants implicated in the etiopathology of several syndromes associated with neutrophil serine proteases, including Papillon–Lefevre syndrome (PLS), Haim–Munk Syndrome (HMS), and aggressive periodontitis (AP). These syndromes are characterized by palmoplantar hyperkeratosis, and early-onset periodontitis (severe gum disease) resulting in premature tooth loss. Due to the critical role played by CTSC in these and several other conditions it is being explored as a potential therapeutic target for autoimmune and inflammatory disorders. The review also discusses in depth the gene variants of CTSC, and in particular their postulated association with chronic obstructive pulmonary disease (COPD), COVID-19, various cancers, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, sudden cardiac death (SCD), atherosclerotic vascular disease, and neuroinflammatory disease. Finally, the therapeutic potential of CTSC across a range of human diseases is discussed.

更新日期：2024-08-21

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