Fibrillin-2 is a key mediator of smooth muscle extracellular matrix homeostasis during mouse tracheal tubulogenesis.
Animals
Embryo, Mammalian
Extracellular Matrix
/ metabolism
Female
Fibrillin-2
/ genetics
Fibronectins
/ metabolism
Gene Expression Regulation, Developmental
Homeostasis
Male
Mice
Mice, Inbred C57BL
Muscle, Smooth
/ cytology
Myocytes, Smooth Muscle
/ cytology
Phenotype
Phosphorylation
Signal Transduction
Trachea
/ cytology
Journal
The European respiratory journal
ISSN: 1399-3003
Titre abrégé: Eur Respir J
Pays: England
ID NLM: 8803460
Informations de publication
Date de publication:
03 2019
03 2019
Historique:
received:
08
05
2018
accepted:
28
11
2018
pubmed:
24
12
2018
medline:
10
10
2020
entrez:
23
12
2018
Statut:
epublish
Résumé
Epithelial tubes, comprised of polarised epithelial cells around a lumen, are crucial for organ function. However, the molecular mechanisms underlying tube formation remain largely unknown. Here, we report on the function of fibrillin (FBN)2, an extracellular matrix (ECM) glycoprotein, as a critical regulator of tracheal tube formation.We performed a large-scale forward genetic screen in mouse to identify regulators of respiratory organ development and disease. We identified
Identifiants
pubmed: 30578393
pii: 13993003.00840-2018
doi: 10.1183/13993003.00840-2018
pii:
doi:
Substances chimiques
Fbn2 protein, mouse
0
Fibrillin-2
0
Fibronectins
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Commentaires et corrections
Type : CommentIn
Informations de copyright
Copyright ©ERS 2019.
Déclaration de conflit d'intérêts
Conflict of interest: W. Yin has nothing to disclose. Conflict of interest: H-T. Kim has nothing to disclose. Conflict of interest: S. Wang has nothing to disclose. Conflict of interest: F. Gunawan has nothing to disclose. Conflict of interest: R. Li has nothing to disclose. Conflict of interest: C. Buettner has nothing to disclose. Conflict of interest: B. Grohmann has nothing to disclose. Conflict of interest: G. Sengle has nothing to disclose. Conflict of interest: D. Sinner has nothing to disclose. Conflict of interest: S. Offermanns has nothing to disclose. Conflict of interest: D.Y.R. Stainier has nothing to disclose.