ArticlePediatric pulmonology2015
MUC5B expression and location in surfactant protein C mutations in children.
Article in Pediatric pulmonology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
4 citing papers in PubMed, 7 citations in OpenAlex.
- XBP1S Regulates MUC5B in a Promoter Variant-Dependent Pathway in Idiopathic Pulmonary Fibrosis Airway Epithelia.American journal of respiratory and critical care medicine · 2019Article
- The Possible Pathogenesis of Idiopathic Pulmonary Fibrosis consideringBioMed research international · 2019Review
- Oxidative and endoplasmic reticulum stress in respiratory disease.Clinical & translational immunology · 2018Review
- Candidate genes of idiopathic pulmonary fibrosis: current evidence and research.The application of clinical genetics · 2016Review
Corrections and comments
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Authors and funding
10 authors at 2 institutions in 1 country.
Funding
Abstract
backgroundMutations in Surfactant Protein C (SFTPC) can lead to fibrotic interstitial lung disease (ILD) with variable phenotypes, especially in children. The sources of phenotype variability are incompletely understood. A common MUC5B promoter variant rs35705950 is associated with adult Idiopathic Pulmonary Fibrosis (IPF). We examined whether MUC5B is similarly linked to ILD secondary to SFTPC mutations.
methodsMUC5B concentration in bronchoalveolar lavage fluid (BALF) was measured in six pediatric patients with SFTPC mutations and diseased controls. Immunohistochemical localization of MUC5B was studied in fixed lung tissues in patients with SFTPC mutations, ABCA3 mutations, and controls. Genotyping for the MUC5B promoter variant rs35705950 was attempted in all samples.
resultsMUC5B glycoprotein was increased in BALF of patients with SFTPC mutations compared to diseased controls (P = 0.04). MUC5B was unexpectedly present in cells morphologically consistent with alveolar epithelial type II cells in patients with SFTPC mutations in the BRICHOS domain. Genotyping for the MUC5B promoter variant was successful in 18/27 patients, and there was no significant relationship between the MUC5B promoter variant and the BALF or MUC5B localization.
conclusionMUC5B may play a role in the development of fibrosis in patients with SFTPC mutations, especially in patients with BRICHOS mutations. Understanding the role of MUC5B in adult and pediatric lung diseases may lead to a better understanding of the etiology of fibrotic lung disease as well as development of novel therapies.
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