https://www.ncbi.nlm.nih.gov/pubmed/31514468
J Clin Med. 2019 Sep 11;8(9). pii: E1447. doi: 10.3390/jcm8091447.
Mucins as a New Frontier in Pulmonary Fibrosis.
Abstract
Idiopathic
pulmonary fibrosis (IPF) is the most common idiopathic interstitial
pulmonary disease with a median survival of 3-5 years after diagnosis.
Recent evidence identifies mucins as key effectors in cell growth and
tissue remodeling processes compatible with the processes observed in
IPF. Mucins are classified in two groups depending on whether they are
secreted (secreted mucins) or tethered to cell membranes (transmembrane
mucins). Secreted mucins (MUC2,
MUC5AC, MUC5B, MUC6-8 and MUC19) are released to the extracellular
medium and recent evidence has shown that a promoter polymorphism in the
secreted mucin MUC5B is associated with IPF risk. Otherwise,
transmembrane mucins (MUC1, MUC3, MUC4,
MUC12-17 and MUC20) have a receptor-like structure, sensing the
external environment and activating intracellular signal transduction
pathways essential for mucosal maintenance and damage repair. In this
context, the extracellular domain can be released to the external
environment by metalloproteinase action, increased in IPF, thus
activating fibrotic processes. For example, several studies have
reported increased serum extracellular secreted KL6/MUC1 during IPF acute exacerbation. Moreover, MUC1 and MUC4
overexpression in the main IPF cells has been observed. In this review
we summarize the current knowledge of mucins as promising druggable
targets for IPF.
https://www.karger.com/Article/Fulltext/442794
https://www.karger.com/Article/Fulltext/442794
Pharmacology 2016;97:84-100
Novel Therapies to Inhibit Mucus Synthesis and Secretion in Airway Hypersecretory DiseasesHa E.V.S. · Rogers D.F.Author affiliations Corresponding Author |
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