title: Proteostasis in pediatric pulmonary pathology.
abstract: 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.
authors.affiliation: Comprehensive Pneumology Center (CPC), Member of the German Center for Lung Research (DZL), University Hospital, Ludwig-Maximilians-Universität, Asklepios Klinik Gauting und Helmholtz Zentrum München, Max-Lebsche-Platz 31, 81377, Munich, Germany. silke.meiners@helmholtz-muenchen.de.
authors.forename: Silke
authors.initials: S
authors.lastname: Meiners
authors.affiliation: Comprehensive Pneumology Center (CPC), Member of the German Center for Lung Research (DZL), University Hospital, Ludwig-Maximilians-Universität, Asklepios Klinik Gauting und Helmholtz Zentrum München, Max-Lebsche-Platz 31, 81377, Munich, Germany. korbinian.ballweg@helmholtz-muenchen.de.
authors.forename: Korbinian
authors.initials: K
authors.lastname: Ballweg
keywords: Autophagy; Conformational disorder; ER stress; Proteasome; Proteostasis