Respiratory defects in the
Crtap
collagen
lung
osteogenesis imperfecta
respiratory function
Journal
American journal of physiology. Lung cellular and molecular physiology
ISSN: 1522-1504
Titre abrégé: Am J Physiol Lung Cell Mol Physiol
Pays: United States
ID NLM: 100901229
Informations de publication
Date de publication:
01 04 2020
01 04 2020
Historique:
pubmed:
6
2
2020
medline:
17
7
2020
entrez:
6
2
2020
Statut:
ppublish
Résumé
Respiratory disease is a leading cause of mortality in patients with osteogenesis imperfecta (OI), a connective tissue disease that causes severely reduced bone mass and is most commonly caused by dominant mutations in type I collagen genes. Previous studies proposed that impaired respiratory function in OI patients was secondary to skeletal deformities; however, recent evidence suggests the existence of a primary lung defect. Here, we analyzed the lung phenotype of
Identifiants
pubmed: 32022592
doi: 10.1152/ajplung.00313.2019
pmc: PMC7191481
doi:
Substances chimiques
Collagen Type I
0
Crtap protein, mouse
0
Extracellular Matrix Proteins
0
Molecular Chaperones
0
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Langues
eng
Sous-ensembles de citation
IM
Pagination
L592-L605Subventions
Organisme : NIGMS NIH HHS
ID : P20 GM121293
Pays : United States
Organisme : NIGMS NIH HHS
ID : P20 GM125503
Pays : United States
Organisme : NICHD NIH HHS
ID : R03 HD097559
Pays : United States
Organisme : NCATS NIH HHS
ID : U54 TR001629
Pays : United States
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