RNA sequencing of transplant-stage idiopathic pulmonary fibrosis lung reveals unique pathway regulation.
Journal
ERJ open research
ISSN: 2312-0541
Titre abrégé: ERJ Open Res
Pays: England
ID NLM: 101671641
Informations de publication
Date de publication:
Jul 2019
Jul 2019
Historique:
received:
13
05
2019
accepted:
15
06
2019
entrez:
20
8
2019
pubmed:
20
8
2019
medline:
20
8
2019
Statut:
epublish
Résumé
Idiopathic pulmonary fibrosis (IPF), the scarring of lung parenchyma resulting in the loss of lung function, remains a fatal disease with a significant unmet medical need. Patients with severe IPF often develop acute exacerbations resulting in the rapid deterioration of lung function, requiring transplantation. Understanding the pathophysiological mechanisms contributing to IPF is key to develop novel therapeutic approaches for end-stage disease. We report here RNA-sequencing analyses of lung tissues from a cohort of patients with transplant-stage IPF (n=36), compared with acute lung injury (ALI) (n=11) and nondisease controls (n=19), that reveal a robust gene expression signature unique to end-stage IPF. In addition to extracellular matrix remodelling pathways, we identified pathways associated with T-cell infiltration/activation, tumour development, and cholesterol homeostasis, as well as novel alternatively spliced transcripts that are differentially regulated in the advanced IPF lung
Identifiants
pubmed: 31423451
doi: 10.1183/23120541.00117-2019
pii: 00117-2019
pmc: PMC6689672
pii:
doi:
Types de publication
Journal Article
Langues
eng
Subventions
Organisme : NIEHS NIH HHS
ID : P30 ES013508
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL074064
Pays : United States
Déclaration de conflit d'intérêts
Conflict of interest: P. Sivakumar has nothing to disclose. Conflict of interest: J.R. Thompson has nothing to disclose. Conflict of interest: R. Ammar has nothing to disclose. Conflict of interest: M. Porteous has nothing to disclose. Conflict of interest: C. McCoubrey has nothing to disclose. Conflict of interest: E. Cantu III has nothing to disclose. Conflict of interest: K. Ravi has nothing to disclose. Conflict of interest: Y. Zhang has nothing to disclose. Conflict of interest: Y. Luo has nothing to disclose. Conflict of interest: D. Streltsov has nothing to disclose. Conflict of interest: M.F. Beers reports grants from Bristol-Myers Squibb during the conduct of the study. Conflict of interest: G. Jarai has nothing to disclose. Conflict of interest: J.D. Christie reports grants from the NIH and Bristol-Myers Squibb during the conduct of the study and grants from the NIH, GlaxoSmithKline and Bristol-Myers Squibb outside the submitted work.
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