Developmental pathways in the pathogenesis of lung fibrosis.
Antagonistic pleiotropy
Development
Fibroblast growth factor (FGF)
Fibrosis
Hippo
Lung
Notch
Platelet derived growth factor (PDGF)
Sonic hedgehog (SHH)
Transforming growth factor-β (TGF-β)
Wnt
Journal
Molecular aspects of medicine
ISSN: 1872-9452
Titre abrégé: Mol Aspects Med
Pays: England
ID NLM: 7603128
Informations de publication
Date de publication:
02 2019
02 2019
Historique:
received:
26
07
2018
accepted:
17
08
2018
pubmed:
22
8
2018
medline:
13
7
2019
entrez:
22
8
2018
Statut:
ppublish
Résumé
Idiopathic pulmonary fibrosis (IPF) is a progressive and terminal lung disease with no known cure. IPF is a disease of aging, with median age of diagnosis over 65 years. Median survival is between 3 and 5 years after diagnosis. IPF is characterized primarily by excessive deposition of extracellular matrix (ECM) proteins by activated lung fibroblasts and myofibroblasts, resulting in reduced gas exchange and impaired pulmonary function. Growing evidence supports the concept of a pro-fibrotic environment orchestrated by underlying factors such as genetic predisposition, chronic injury and aging, oxidative stress, and impaired regenerative responses may account for disease development and persistence. Currently, two FDA approved drugs have limited efficacy in the treatment of IPF. Many of the genes and gene networks associated with lung development are induced or activated in IPF. In this review, we analyze current knowledge in the field, gained from both basic and clinical research, to provide new insights into the disease process, and potential approaches to treatment of pulmonary fibrosis.
Identifiants
pubmed: 30130563
pii: S0098-2997(18)30071-2
doi: 10.1016/j.mam.2018.08.004
pmc: PMC6374163
mid: NIHMS1504871
pii:
doi:
Substances chimiques
Biomarkers
0
Homeodomain Proteins
0
Trans-Activators
0
Transforming Growth Factor beta
0
pancreatic and duodenal homeobox 1 protein
0
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, U.S. Gov't, Non-P.H.S.
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
56-69Subventions
Organisme : NHLBI NIH HHS
ID : R01 HL132156
Pays : United States
Organisme : BLRD VA
ID : IK2 BX001477
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL126732
Pays : United States
Organisme : NHLBI NIH HHS
ID : P01 HL114470
Pays : United States
Organisme : NHLBI NIH HHS
ID : T32 HL134640
Pays : United States
Organisme : NIA NIH HHS
ID : R01 AG046210
Pays : United States
Organisme : BLRD VA
ID : I01 BX003056
Pays : United States
Informations de copyright
Copyright © 2018. Published by Elsevier Ltd.
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