关键词: Cathepsin C Cathepsin G Inflammation Neutrophil elastase Neutrophil serine proteinase Proteinase 3

Mesh : Humans Cathepsin C / genetics Papillon-Lefevre Disease / genetics drug therapy Lung Diseases Myeloblastin Mutation Neutrophils

来  源:   DOI:10.1016/j.biochi.2023.09.018

Abstract:
Cathepsin C (CatC, syn. Dipeptidyl peptidase I) is a lysosomal cysteine proteinase expressed in several tissues including inflammatory cells. This enzyme is important for maintaining multiple cellular functions and for processing immune cell-derived proteases. While mutations in the CatC gene were reported in Papillon-Lefèvre syndrome, a rare autosomal recessive disorder featuring hyperkeratosis and periodontitis, evidence from clinical and preclinical studies points toward pro-inflammatory effects of CatC in various disease processes that are mainly mediated by the activation of neutrophil serine proteinases. Moreover, tumor-promoting effects were ascribed to CatC. The aim of this review is to highlight current knowledge of the CatC as a potential therapeutic target in inflammatory disorders.
摘要:
组织蛋白酶C(CatC,syn.二肽基肽酶I)是在包括炎性细胞在内的几种组织中表达的溶酶体半胱氨酸蛋白酶。该酶对于维持多种细胞功能和加工免疫细胞来源的蛋白酶是重要的。虽然在Papillon-Lefèvre综合征中报道了CatC基因的突变,一种罕见的常染色体隐性遗传疾病,以角化过度和牙周炎为特征,来自临床和临床前研究的证据表明,CatC在各种疾病过程中的促炎作用主要由中性粒细胞丝氨酸蛋白酶的激活介导。此外,促肿瘤作用归因于CatC。这篇综述的目的是强调目前关于CatC作为炎症性疾病潜在治疗靶点的知识。
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