Patient-specific iPSCs carrying an SFTPC mutation reveal the intrinsic alveolar epithelial dysfunction at the inception of interstitial lung disease.
Alveolar Epithelial Cells
/ metabolism
Animals
Cell Line
Cell Proliferation
Energy Metabolism
Genetic Predisposition to Disease
Humans
Induced Pluripotent Stem Cells
/ metabolism
Inflammation Mediators
/ metabolism
Lung Diseases, Interstitial
/ genetics
Mice, Knockout
Mutation
NF-kappa B
/ metabolism
Phenotype
Proteostasis
Pulmonary Surfactant-Associated Protein C
/ genetics
Signal Transduction
NF-κB
autophagy
bioenergetics
iPSC-derived alveolar epithelial type 2 cells
idiopathic pulmonary fibrosis
induced pluripotent stem cells
interstitial lung disease
metabolic reprogramming
proteostasis
surfactant protein C
Journal
Cell reports
ISSN: 2211-1247
Titre abrégé: Cell Rep
Pays: United States
ID NLM: 101573691
Informations de publication
Date de publication:
31 08 2021
31 08 2021
Historique:
received:
18
11
2020
revised:
28
04
2021
accepted:
06
08
2021
entrez:
1
9
2021
pubmed:
2
9
2021
medline:
15
2
2022
Statut:
ppublish
Résumé
Alveolar epithelial type 2 cell (AEC2) dysfunction is implicated in the pathogenesis of adult and pediatric interstitial lung disease (ILD), including idiopathic pulmonary fibrosis (IPF); however, identification of disease-initiating mechanisms has been impeded by inability to access primary AEC2s early on. Here, we present a human in vitro model permitting investigation of epithelial-intrinsic events culminating in AEC2 dysfunction, using patient-specific induced pluripotent stem cells (iPSCs) carrying an AEC2-exclusive disease-associated variant (SFTPC
Identifiants
pubmed: 34469722
pii: S2211-1247(21)01079-2
doi: 10.1016/j.celrep.2021.109636
pmc: PMC8432578
mid: NIHMS1737383
pii:
doi:
Substances chimiques
Inflammation Mediators
0
NF-kappa B
0
Pulmonary Surfactant-Associated Protein C
0
SFTPC protein, human
0
Sftpc protein, mouse
0
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, Non-P.H.S.
Langues
eng
Sous-ensembles de citation
IM
Pagination
109636Subventions
Organisme : NHLBI NIH HHS
ID : R01 HL119436
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL150617
Pays : United States
Organisme : NCATS NIH HHS
ID : U01 TR001810
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL149853
Pays : United States
Organisme : NHLBI NIH HHS
ID : U01 HL152976
Pays : United States
Organisme : NHLBI NIH HHS
ID : U01 HL152970
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL145408
Pays : United States
Organisme : NIEHS NIH HHS
ID : P30 ES013508
Pays : United States
Organisme : BLRD VA
ID : I01 BX001176
Pays : United States
Organisme : NCATS NIH HHS
ID : UL1 TR001430
Pays : United States
Organisme : NHLBI NIH HHS
ID : K24 HL143281
Pays : United States
Organisme : NHLBI NIH HHS
ID : U01 HL134745
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL095993
Pays : United States
Organisme : NIAAA NIH HHS
ID : R01 AA026914
Pays : United States
Organisme : NHLBI NIH HHS
ID : U01 HL134766
Pays : United States
Organisme : NHLBI NIH HHS
ID : U01 HL148692
Pays : United States
Informations de copyright
Copyright © 2021 The Author(s). Published by Elsevier Inc. All rights reserved.
Déclaration de conflit d'intérêts
Declaration of interests The authors declare no competing interests.
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