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Open Access Gold as soon as Publ. Version/Full Text is submitted to ZB.
Proteostasis in pediatric pulmonary pathology.
Mol. Cell. Pediatr. 1:11 (2014)
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.
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Publication type
Article: Journal article
Document type
Scientific Article
Keywords
Autophagy ; Conformational Disorder ; Er Stress ; Proteasome ; Proteostasis
ISSN (print) / ISBN
2194-7791
Quellenangaben
Volume: 1,
Article Number: 11
Publisher
Springer
Publishing Place
Berlin ; Heidelberg [u.a.]
Non-patent literature
Publications
Reviewing status
Peer reviewed
Institute(s)
Institute of Lung Health and Immunity (LHI)