A surfactant protein C precursor protein BRICHOS domain mutation causes endoplasmic reticulum stress, proteasome dysfunction, and caspase 3 activation
about
ERdj4 and ERdj5 are required for endoplasmic reticulum-associated protein degradation of misfolded surfactant protein CMeckel-Gruber syndrome protein MKS3 is required for endoplasmic reticulum-associated degradation of surfactant protein CGenetic interstitial lung diseaseInterstitial Lung Disease in Childhood: Clinical and Genetic AspectsUnderstanding Idiopathic Interstitial Pneumonia: A Gene-Based Review of Stressed LungsEMT and interstitial lung disease: a mysterious relationshipInterstitial lung disease in childrenCellular mechanisms of tissue fibrosis. 7. New insights into the cellular mechanisms of pulmonary fibrosisMolecular biomarkers in idiopathic pulmonary fibrosisGenetic disorders of surfactant dysfunctionThe Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segmentHuman surfactant protein A2 gene mutations impair dimmer/trimer assembly leading to deficiency in protein sialylation and secretionC-terminal, endoplasmic reticulum-lumenal domain of prosurfactant protein C - structural features and membrane interactions.Genetic defects in surfactant protein A2 are associated with pulmonary fibrosis and lung cancer.Epithelial endoplasmic reticulum stress and apoptosis in sporadic idiopathic pulmonary fibrosis.Gastrokines: stomach-specific proteins with putative homeostatic and tumor suppressor roles.Fibrosis of two: Epithelial cell-fibroblast interactions in pulmonary fibrosisA non-BRICHOS surfactant protein c mutation disrupts epithelial cell function and intercellular signalingPulmonary alveolar proteinosis in children on La Réunion Island: a new inherited disorder?Identification of early interstitial lung disease in an individual with genetic variations in ABCA3 and SFTPC.Surfactant protein A2 mutations associated with pulmonary fibrosis lead to protein instability and endoplasmic reticulum stress.Protein C mutation (A267T) results in ER retention and unfolded protein response activation.Pathogenesis of idiopathic pulmonary fibrosis: review of recent findings.Epithelial stress and apoptosis underlie Hermansky-Pudlak syndrome-associated interstitial pneumoniaThe surfactant protein C mutation A116D alters cellular processing, stress tolerance, surfactant lipid composition, and immune cell activation.Mutant surfactant A2 proteins associated with familial pulmonary fibrosis and lung cancer induce TGF-β1 secretion.Diseases of pulmonary surfactant homeostasis.Heterozygosity for ABCA3 mutations modifies the severity of lung disease associated with a surfactant protein C gene (SFTPC) mutation.A non-BRICHOS SFTPC mutant (SP-CI73T) linked to interstitial lung disease promotes a late block in macroautophagy disrupting cellular proteostasis and mitophagyGenetics in pulmonary fibrosis--familial cases provide clues to the pathogenesis of idiopathic pulmonary fibrosis.The three R's of lung health and disease: repair, remodeling, and regeneration.Endoplasmic reticulum stress enhances fibrotic remodeling in the lungsIncreased and prolonged pulmonary fibrosis in surfactant protein C-deficient mice following intratracheal bleomycinExtensive phenotyping of individuals at risk for familial interstitial pneumonia reveals clues to the pathogenesis of interstitial lung disease.A nonaggregating surfactant protein C mutant is misdirected to early endosomes and disrupts phospholipid recycling.Alveolar epithelial cells undergo epithelial-to-mesenchymal transition in response to endoplasmic reticulum stressRole of endoplasmic reticulum stress in epithelial-mesenchymal transition of alveolar epithelial cells: effects of misfolded surfactant protein.Surfactant dysfunction.Genetic Basis of Children's Interstitial Lung Disease.Peptide-based interactions with calnexin target misassembled membrane proteins into endoplasmic reticulum-derived multilamellar bodies.
P2860
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P2860
A surfactant protein C precursor protein BRICHOS domain mutation causes endoplasmic reticulum stress, proteasome dysfunction, and caspase 3 activation
description
2005 nî lūn-bûn
@nan
2005 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
2005 թվականի հունիսին հրատարակված գիտական հոդված
@hy
2005年の論文
@ja
2005年論文
@yue
2005年論文
@zh-hant
2005年論文
@zh-hk
2005年論文
@zh-mo
2005年論文
@zh-tw
2005年论文
@wuu
name
A surfactant protein C precurs ...... tion, and caspase 3 activation
@ast
A surfactant protein C precurs ...... tion, and caspase 3 activation
@en
A surfactant protein C precurs ...... tion, and caspase 3 activation
@nl
type
label
A surfactant protein C precurs ...... tion, and caspase 3 activation
@ast
A surfactant protein C precurs ...... tion, and caspase 3 activation
@en
A surfactant protein C precurs ...... tion, and caspase 3 activation
@nl
prefLabel
A surfactant protein C precurs ...... tion, and caspase 3 activation
@ast
A surfactant protein C precurs ...... tion, and caspase 3 activation
@en
A surfactant protein C precurs ...... tion, and caspase 3 activation
@nl
P2093
P2860
P356
P1476
A surfactant protein C precurs ...... tion, and caspase 3 activation
@en
P2093
Madesh Muniswamy
Michael F Beers
Scott J Russo
Surafel Mulugeta
P2860
P304
P356
10.1165/RCMB.2005-0009OC
P407
P577
2005-06-01T00:00:00Z