A nonaggregating surfactant protein C mutant is misdirected to early endosomes and disrupts phospholipid recycling.
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Understanding Idiopathic Interstitial Pneumonia: A Gene-Based Review of Stressed LungsA non-BRICHOS SFTPC mutant (SP-CI73T) linked to interstitial lung disease promotes a late block in macroautophagy disrupting cellular proteostasis and mitophagyLost after translation: insights from pulmonary surfactant for understanding the role of alveolar epithelial dysfunction and cellular quality control in fibrotic lung disease.4-Phenylbutyric acid treatment rescues trafficking and processing of a mutant surfactant protein-C.Surfactant Protein C-associated interstitial lung disease; three different phenotypes of the same SFTPC mutation.Clinical and ultrastructural spectrum of diffuse lung disease associated with surfactant protein C mutations.Lung fibrosis-associated surfactant protein A1 and C variants induce latent transforming growth factor β1 secretion in lung epithelial cells.Disruption of N-linked glycosylation promotes proteasomal degradation of the human ATP-binding cassette transporter ABCA3.A novel surfactant protein C L55F mutation associated with interstitial lung disease alters subcellular localization of proSP-C in A549 cells.The genetic basis of idiopathic pulmonary fibrosisFolding and Intramembraneous BRICHOS Binding of the Prosurfactant Protein C Transmembrane SegmentSuccessful treatment of neonatal respiratory failure caused by a novel surfactant protein C p.Cys121Gly mutation with hydroxychloroquine.SFTPC mutations cause SP-C degradation and aggregate formation without increasing ER stress.EMC3 coordinates surfactant protein and lipid homeostasis required for respiration.Deciphering the mechanism of Q145H SFTPC mutation unmasks a splicing defect and explains the severity of the phenotype.Linking progression of fibrotic lung remodeling and ultrastructural alterations of alveolar epithelial type II cells in the amiodarone mouse model.
P2860
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P2860
A nonaggregating surfactant protein C mutant is misdirected to early endosomes and disrupts phospholipid recycling.
description
2011 nî lūn-bûn
@nan
2011 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
2011 թվականի հունիսին հրատարակված գիտական հոդված
@hy
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
name
A nonaggregating surfactant pr ...... srupts phospholipid recycling.
@ast
A nonaggregating surfactant pr ...... srupts phospholipid recycling.
@en
type
label
A nonaggregating surfactant pr ...... srupts phospholipid recycling.
@ast
A nonaggregating surfactant pr ...... srupts phospholipid recycling.
@en
prefLabel
A nonaggregating surfactant pr ...... srupts phospholipid recycling.
@ast
A nonaggregating surfactant pr ...... srupts phospholipid recycling.
@en
P2093
P2860
P1433
P1476
A nonaggregating surfactant pr ...... isrupts phospholipid recycling
@en
P2093
Adam Kotorashvili
Arie Hawkins
Jean Ann Maguire
Jennifer L Newitt
Linda Gonzales
Michael F Beers
Scott Russo
Surafel Mulugeta
Wenge Ding
P2860
P304
P356
10.1111/J.1600-0854.2011.01223.X
P577
2011-06-28T00:00:00Z