Cystic Fibrosis Defective Response to Infection Involves Autophagy and Lipid Metabolism.
Antifungal Agents
/ pharmacology
Aspergillosis
/ metabolism
Autophagy
Cell Line
Cystic Fibrosis
/ metabolism
Epithelial Cells
/ metabolism
Fatty Acids, Monounsaturated
/ pharmacology
Gene Expression
/ drug effects
Humans
Inflammation
/ metabolism
Leukocytes, Mononuclear
/ metabolism
Lipid Metabolism
/ drug effects
Sphingolipids
/ metabolism
Aspergillus fumigatus
autophagy
cystic fibrosis
myriocin
sphingolipids
Journal
Cells
ISSN: 2073-4409
Titre abrégé: Cells
Pays: Switzerland
ID NLM: 101600052
Informations de publication
Date de publication:
06 08 2020
06 08 2020
Historique:
received:
26
06
2020
revised:
31
07
2020
accepted:
04
08
2020
entrez:
13
8
2020
pubmed:
13
8
2020
medline:
24
3
2021
Statut:
epublish
Résumé
Cystic fibrosis (CF) is a hereditary disease, with 70% of patients developing a proteinopathy related to the deletion of phenylalanine 508. CF is associated with multiple organ dysfunction, chronic inflammation, and recurrent lung infections. CF is characterized by defective autophagy, lipid metabolism, and immune response. Intracellular lipid accumulation favors microbial infection, and autophagy deficiency impairs internalized pathogen clearance. Myriocin, an inhibitor of sphingolipid synthesis, significantly reduces inflammation, promotes microbial clearance in the lungs, and induces autophagy and lipid oxidation. RNA-seq was performed in
Identifiants
pubmed: 32781626
pii: cells9081845
doi: 10.3390/cells9081845
pmc: PMC7463682
pii:
doi:
Substances chimiques
Antifungal Agents
0
Fatty Acids, Monounsaturated
0
Sphingolipids
0
thermozymocidin
YRM4E8R9ST
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
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