Protein homeostasis describes the tight supervision of protein synthesis, correct protein maturation and folding, as well as the timely disposal of unwanted and damaged proteins by the ubiquitin-proteasome pathway or the lysosome-autophagy route. The cellular processes involved in preservation of protein homeostasis are collectively called proteostasis. Dysregulation of proteostasis is an emerging common pathomechanism for chronic lung diseases in the adult and aged patient. There is also rising evidence that impairment of protein homeostasis contributes to early sporadic disease onset in pediatric lung diseases beyond the well-known hereditary proteostasis disorders such as cystic fibrosis and alpha-1 antitrypsin (AAT) deficiency. Identifying the pathways that contribute to impaired proteostasis will provide new avenues for therapeutic interference with the pathogenesis of chronic lung diseases in the young and adult. Here, we introduce the concept of proteostasis and summarize available evidence on dysregulation of proteostasis pathways in pediatric and adult chronic lung diseases.

中文翻译：

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小儿肺部病理学中的蛋白质稳态

蛋白质稳态描述了对蛋白质合成、正确蛋白质成熟和折叠的严格监督，以及通过泛素-蛋白酶体途径或溶酶体-自噬途径及时处理不需要的和受损的蛋白质。参与维持蛋白质稳态的细胞过程统称为蛋白质稳态。蛋白质稳态失调是成人和老年患者慢性肺病的一种新兴常见病理机制。越来越多的证据表明，除了众所周知的遗传性蛋白质稳态失调（例如囊性纤维化和 α-1 抗胰蛋白酶 (AAT) 缺乏症）之外，蛋白质稳态受损还会导致小儿肺部疾病的早期散发性疾病发作。确定导致蛋白质稳态受损的途径将为治疗干扰年轻人和成人慢性肺病的发病机制提供新的途径。在这里，我们介绍了蛋白质稳态的概念，并总结了有关儿童和成人慢性肺病中蛋白质稳态通路失调的现有证据。